Naczyniakomłoniak
Rokowania, prognozy i postęp choroby

Naczyniakomięśniak (angiosarcoma) to agresywny nowotwór złośliwy wywodzący się z komórek śródbłonka naczyń krwionośnych lub limfatycznych, charakteryzujący się złym rokowaniem i często wieloogniskowym przebiegiem. Mediana całkowitego przeżycia (OS) wynosi od 16 do 19,3 miesiąca, a 5-letnie OS około 40%. Rokowanie jest silnie zależne od stadium choroby: w przypadku choroby przerzutowej 1-roczne OS spada do 20,8%, a 3-letnie do 3,8%. Lokalizacja guza pierwotnego ma kluczowe znaczenie prognostyczne – angiosarcoma trzewny/głębokiej tkanki miękkiej cechuje mediana OS około 5 miesięcy, podczas gdy powierzchowny ma medianę OS około 60 miesięcy. Szczególnie niekorzystne jest rokowanie w pierwotnym angiosarcoma wątroby (mediana OS 1,9 miesiąca) oraz śledziony (5-6 miesięcy). Wielkość guza >5 cm, obecność martwicy oraz wysoki stopień histologiczny znacząco pogarszają przeżycie, podobnie jak choroba przerzutowa w momencie rozpoznania (mediana OS około 3 miesięcy). Wiek, płeć, stan sprawności ECOG oraz parametry laboratoryjne (bilirubina, albumina) również wpływają na rokowanie.

Naczyniakomięśniak (Angiosarcoma) – Prognoza i przewidywanie wyników leczenia

Naczyniakomięśniak (angiosarcoma) to agresywny nowotwór złośliwy wywodzący się z komórek śródbłonka naczyń krwionośnych (hemangiosarcoma) lub limfatycznych (lymphangiosarcoma). Charakteryzuje się wyjątkowo złym rokowaniem pomimo intensywnego leczenia. Nowotwór ten jest klinicznie agresywny, trudny w leczeniu i często występuje w postaci wieloogniskowej.123

Wskaźniki przeżycia

Dane dotyczące wskaźników przeżycia w naczyniakomięśniaku wskazują na generalnie złe rokowanie:456

  • Mediana całkowitego przeżycia (OS) wynosi od 16 do 19,3 miesiąca78
  • 5-letnie całkowite przeżycie (OS) wynosi około 40%910
  • Wskaźniki 1-rocznego OS wynoszą około 55-57,5%1112
  • Wskaźniki 3-letniego OS wynoszą około 33,7%13
  • Przeżycie wolne od choroby (DFS) w ciągu 5 lat wynosi około 19,3%14

Dane dotyczące przeżycia różnią się znacząco w zależności od stadium choroby w momencie rozpoznania. W przypadku choroby rozsianej (przerzutowej) rokowanie jest szczególnie niekorzystne:15

  • 1-roczne OS w chorobie przerzutowej: 20,8%16
  • 3-letnie OS w chorobie przerzutowej: 3,8%17
  • 1-roczne przeżycie specyficzne dla nowotworu (CSS) w chorobie przerzutowej: 22,0%18
  • 3-letnie CSS w chorobie przerzutowej: 5,2%19

Czynniki prognostyczne związane z guzem

Lokalizacja guza pierwotnego jest jednym z kluczowych czynników wpływających na rokowanie:202122

  • Angiosarcoma trzewny/głębokiej tkanki miękkiej: mediana OS około 5 miesięcy23
  • Angiosarcoma nietrzewny/powierzchowny: mediana OS około 60 miesięcy24
  • Pierwotny angiosarcoma wątroby: szczególnie złe rokowanie z medianą przeżycia 1,9 miesiąca (7,7 miesiąca po resekcji chirurgicznej)25
  • Angiosarcoma śledziony: mediana OS 5-6 miesięcy (do 14 miesięcy po wczesnej splenektomii)26
  • Angiosarcoma piersi: względnie lepsze rokowanie, z 5-letnim OS sięgającym 87,5%27
  • Angiosarcoma głowy i szyi: gorsze rokowanie, z 5-letnim OS około 28,0%28

Wielkość guza jest istotnym czynnikiem prognostycznym, co potwierdzają liczne badania:293031

  • Guzy o średnicy ≤5 cm: mediana OS około 60 miesięcy32
  • Guzy o średnicy >5 cm: mediana OS około 10 miesięcy33

Stopień zaawansowania i cechy histologiczne również mają znaczący wpływ na rokowanie:343536

  • Obecność martwicy w guzie: mediana OS 9 miesięcy37
  • Brak martwicy w guzie: mediana OS 60 miesięcy38
  • Przeżycie wolne od choroby w zależności od stopnia złośliwości histologicznej: 95% w stopniu 1, 62% w stopniu 2, 20% w stopniu 339

Status choroby w momencie rozpoznania jest kluczowym czynnikiem prognostycznym:4041

  • Choroba przerzutowa w momencie rozpoznania: mediana OS około 3 miesięcy42
  • Choroba zlokalizowana: mediana OS około 60 miesięcy43
  • Choroba nawrotowa miejscowo w momencie rozpoznania: istotnie zwiększone ryzyko miejscowej progresji (HR: 5,32, p=0,013)44

Biomarkery i czynniki molekularne

Badania nad biomarkerami dostarczają dodatkowych informacji prognostycznych dla pacjentów z angiosarcoma:4546

Czynniki demograficzne i kliniczne

W analizach wieloczynnikowych zidentyfikowano następujące czynniki prognostyczne:515253

  • Wiek: każdy dodatkowy rok zwiększa ryzyko zgonu (HR: 1,03, p=0,044) i miejscowej progresji (HR: 1,04, p=0,008)54
  • Płeć: czynnik niezależnie związany z rokowaniem5556
  • Stan sprawności wg ECOG: gorszy stan sprawności wiąże się z gorszym rokowaniem57
  • Parametry laboratoryjne: wyższy poziom bilirubiny i niższy poziom albuminy przed chemioterapią wiążą się z gorszym OS58

Wpływ leczenia na rokowanie

Wybór metody leczenia ma istotny wpływ na rokowanie pacjentów z naczyniakomięśniakiem:596061

  • Leczenie chirurgiczne: kluczowa rola w poprawie rokowania, szczególnie w chorobie zlokalizowanej6263
  • Chemioterapia: przynosi korzyści w chorobie przerzutowej, z paklitakselem w schemacie cotygodniowym jako preferowaną opcją646566
  • Radioterapia: niezależny korzystny czynnik predykcyjny OS67
  • Leczenie skojarzone: w niektórych badaniach nie wykazano istotnych korzyści w porównaniu z samą chirurgią68
  • Dodanie bewacyzumabu do cotygodniowego paklitakselu nie poprawiło przeżycia wolnego od progresji (PFS) w zaawansowanym angiosarcoma69

Modele prognostyczne i nomogramy

Ostatnie badania doprowadziły do opracowania nomogramów predykcyjnych, które mogą pomóc w dokładniejszym przewidywaniu rokowania:707172

  • Opracowane nomogramy wykazują dobrą dokładność predykcyjną z indeksem zgodności (C-index) powyżej 0,747374
  • Modele te uwzględniają wiele czynników, w tym płeć, wiek, stopień zaawansowania wg AJCC, stopień histologiczny, lokalizację guza pierwotnego oraz wielkość guza75
  • Nomogramy mogą zapewnić lepszą stratyfikację ryzyka niż tradycyjne systemy oceny stopnia zaawansowania AJCC76

Implikacje kliniczne dla praktyki medycznej

Znajomość czynników prognostycznych pozwala na lepsze planowanie leczenia i informowanie pacjentów:777879

  • Wczesne wykrycie i radykalne wycięcie chirurgiczne są kluczowe dla poprawy rokowania8081
  • Pacjenci z chorobą przerzutową wymagają intensywnej chemioterapii, choć rokowanie pozostaje złe8283
  • Leczenie wielomodalne może poprawić wyniki kliniczne84
  • Potrzebne są nowe metody terapeutyczne, szczególnie dla pacjentów z chorobą przerzutową8586

Specyficzne typy naczyniakomięśniaka i ich rokowanie

Różne podtypy naczyniakomięśniaka charakteryzują się odmiennym rokowaniem:8788

Angiosarcoma de novo i indukowany

Angiosarcoma de novo wiąże się z gorszym rokowaniem niż angiosarcoma indukowany radioterapią (HR=2,5; p=0,024).8990 Przy leczeniu cotygodniowym paklitakselem, wyniki pacjentów z angiosarcoma indukowanym radioterapią są lepsze niż w przypadku angiosarcoma de novo.91

Angiosarcoma piersi

Angiosarcoma piersi jest często bardziej agresywny niż inne typy nowotworów piersi, jednak w porównaniu z innymi lokalizacjami angiosarcoma, charakteryzuje się stosunkowo lepszym rokowaniem, z 5-letnim OS sięgającym 87,5%.929394

Angiosarcoma wątroby

Pierwotny angiosarcoma wątroby wiąże się ze szczególnie złym rokowaniem. Mediana przeżycia wynosi zaledwie 1,9 miesiąca, choć resekcja chirurgiczna może wydłużyć ją do 7,7 miesiąca, a chemioterapia do 5,1 miesiąca.95 Zajęcie wątroby jest istotnym negatywnym czynnikiem prognostycznym.96

Angiosarcoma śledziony

Pierwotny angiosarcoma śledziony (PAS) to wyjątkowo rzadki i agresywny nowotwór z wysokim ryzykiem przerzutów (70%-85%). Rokowanie jest niekorzystne, z medianą OS wynoszącą 5-6 miesięcy. Wczesna splenektomia może zwiększyć medianę OS do 14 miesięcy.97

Lokalizacja angiosarcoma Mediana przeżycia 5-letnie OS Czynniki poprawiające rokowanie
Angiosarcoma zlokalizowany 60 miesięcy 40% Radykalna resekcja, wielkość ≤5 cm, brak martwicy
Angiosarcoma przerzutowy 3 miesiące 3,8% Chemioterapia, wielkość guza ≤10 cm
Angiosarcoma piersi Nie określono 87,5% Resekcja chirurgiczna, niski stopień złośliwości
Angiosarcoma głowy i szyi Nie określono 28,0% Wczesne wykrycie, radykalna resekcja
Angiosarcoma wątroby 1,9 miesiąca Nie określono Resekcja chirurgiczna (7,7 miesiąca), chemioterapia (5,1 miesiąca)
Angiosarcoma śledziony 5-6 miesięcy Nie określono Wczesna splenektomia (14 miesięcy)

Wnioski

Naczyniakomięśniak (angiosarcoma) pozostaje nowotworem o generalnie złym rokowaniu, szczególnie w stadium zaawansowanym lub przerzutowym.9899 Kluczowe znaczenie dla określenia rokowania mają:100101

  • Lokalizacja guza pierwotnego (szczególnie niekorzystne rokowanie w lokalizacji trzewnej)
  • Stadium choroby w momencie rozpoznania (przerzuty znacząco pogarszają rokowanie)
  • Wielkość guza (guzy >5 cm mają gorsze rokowanie)
  • Czynniki histologiczne (stopień złośliwości, obecność martwicy)
  • Wiek pacjenta (starszy wiek pogarsza rokowanie)
  • Możliwość przeprowadzenia radykalnej resekcji chirurgicznej

Mimo intensywnego leczenia skojarzonego, wyniki pozostają niezadowalające, co podkreśla potrzebę opracowania nowych strategii terapeutycznych.102103 Opracowane nomogramy prognostyczne mogą pomóc w lepszej stratyfikacji ryzyka i planowaniu leczenia dla pacjentów z tym rzadkim i agresywnym nowotworem.104105

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  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #2 Angiosarcoma – Rare Cancers Australia
    http://knowledge.rarecancers.org.au/knowledgebase/cancer-types/237/angiosarcoma
    Angiosarcomas are rare types of sarcomas (cancers arising from bone or soft tissue) that develop in the lining of blood vessels (haemangiosarcoma) or lymph vessels (lymphangiosarcomas) throughout the body. […] It is often aggressive, and may not carry as good of a prognosis as other types of cancer. […] These cancers can often be misdiagnosed, are often aggressive, and may not have as good of a prognosis as other types of angiosarcoma. […] Breast angiosarcomas are often more aggressive than other types of breast cancers, and may not have as good of a prognosis as other breast cancers. […] Angiosarcomas of deep soft tissues are often aggressive, and can have varied prognoses based on tumour location. […] Radiation-induced angiosarcoma can be aggressive, and may not have as good of a prognosis as other types of angiosarcoma.
  • #3 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/276512-overview
    All angiosarcomas tend to be aggressive and are often multicentric. These tumors have high rates of local recurrence and metastasis because of their intrinsic biologic properties and because they are often misdiagnosed, leading to a poor prognosis and a high mortality rate. Malignant vascular tumors are clinically aggressive, difficult to treat, and have a reported 5-year survival rate around 20-35%. […] Advanced stage at presentation and lack of radical or wide excision are associated with higher recurrence rates, higher distant metastasis rates, and worsened survival. This is in contrast to the 5-year survival for all types of soft tissue sarcomas, which is around 65%. […] Angiosarcoma of the viscera (particularly liver and heart) and retroperitoneal disease are especially associated with poor outcome.
  • #4 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #5
    https://journals.lww.com/md-journal/fulltext/2025/01030/trends_in_the_incidence,_survival,_and_prognostic.53.aspx
    This study aimed to investigate the epidemiological trends of angiosarcoma and to establish a tool to estimate its prognosis. […] The median survival time is approximately 30 to 50 months, with a 5-year survival rate of 10% to 50%. […] In multivariable analyses, age, sex, marital status, grade, historical stage, surgery, site, and tumor size were independent prognostic factors for angiosarcoma. […] The concordance index of the nomogram was significantly higher than that of the American Joint Committee on Cancer (AJCC) 6th edition and the AJCC 7th edition (0.74 vs 0.61 vs 0.66, respectively). […] The median OS was 16 months and the 1-, 3-, and 5-year survival rates were 55.2%, 33.7%, and 26.4%, respectively. […] In general, patients with local and stage G1 disease have better survival rates. Overall, patients benefited from surgery (HR, 1.73; 95% CI: 1.5971.874; P.05). […] The nomogram showed a good prediction accuracy. […] The C-index of the nomogram for OS was 0.74 (95% CI: 0.730.75). […] The C-index of our nomogram was significantly better than those of the 6th and 7th editions of the AJCC staging system, indicating the superiority of our nomogram.
  • #6 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    Angiosarcoma is a rare soft tissue sarcoma, and the data about its clinicopathological features and prognostic factors are limited. […] Of the 113 patients with followup data, 46 patients succumbed to the disease with a median interval of 10 months. Tumor recurrence/metastasis was identified in 66 patients with a median interval of 4 months. The disease-free survival (DFS) rate at 5 years was 19.3% and the overall survival (OS) rate at 5 years was 40.8%. […] The prognosis for angiosarcoma is also associated with the primary site of the tumor. Fayette et al retrospectively analyzed the clinicopathological features of 161 cases of angiosarcoma, and demonstrated that the patients’ survival was significantly impacted by the primary site. A primary site in the liver or heart was associated with a poor prognosis, whereas primary soft tissue tumors were associated with a relatively improved prognosis.
  • #7 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #8 Angiosarcoma: clinical outcomes and prognostic factors, a single-center analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC11196347/
    The median OS of 19.3 months and 1 year OS of 57.5% reported herein are comparable to previous cohort studies on localized and metastatic AS. […] In our study, combination therapies (surgery with RT or surgery with CTX vs. surgery alone) were not significantly associated with OS or LPFS. […] In our cohort, locally recurrent disease at presentation was significantly associated with LPFS and not significantly associated with OS. […] Tumor size of 5 cm is repeatedly described as another adverse prognostic factor for OS and disease-specific survival in AS patients. […] Older age and metastatic disease at initial presentation status were adverse prognostic factors for local recurrence and OS in our study cohort.
  • #9 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #10 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    Angiosarcoma is a rare soft tissue sarcoma, and the data about its clinicopathological features and prognostic factors are limited. […] Of the 113 patients with followup data, 46 patients succumbed to the disease with a median interval of 10 months. Tumor recurrence/metastasis was identified in 66 patients with a median interval of 4 months. The disease-free survival (DFS) rate at 5 years was 19.3% and the overall survival (OS) rate at 5 years was 40.8%. […] The prognosis for angiosarcoma is also associated with the primary site of the tumor. Fayette et al retrospectively analyzed the clinicopathological features of 161 cases of angiosarcoma, and demonstrated that the patients’ survival was significantly impacted by the primary site. A primary site in the liver or heart was associated with a poor prognosis, whereas primary soft tissue tumors were associated with a relatively improved prognosis.
  • #11 Angiosarcoma: clinical outcomes and prognostic factors, a single-center analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC11196347/
    The median OS of 19.3 months and 1 year OS of 57.5% reported herein are comparable to previous cohort studies on localized and metastatic AS. […] In our study, combination therapies (surgery with RT or surgery with CTX vs. surgery alone) were not significantly associated with OS or LPFS. […] In our cohort, locally recurrent disease at presentation was significantly associated with LPFS and not significantly associated with OS. […] Tumor size of 5 cm is repeatedly described as another adverse prognostic factor for OS and disease-specific survival in AS patients. […] Older age and metastatic disease at initial presentation status were adverse prognostic factors for local recurrence and OS in our study cohort.
  • #12
    https://journals.lww.com/md-journal/fulltext/2025/01030/trends_in_the_incidence,_survival,_and_prognostic.53.aspx
    This study aimed to investigate the epidemiological trends of angiosarcoma and to establish a tool to estimate its prognosis. […] The median survival time is approximately 30 to 50 months, with a 5-year survival rate of 10% to 50%. […] In multivariable analyses, age, sex, marital status, grade, historical stage, surgery, site, and tumor size were independent prognostic factors for angiosarcoma. […] The concordance index of the nomogram was significantly higher than that of the American Joint Committee on Cancer (AJCC) 6th edition and the AJCC 7th edition (0.74 vs 0.61 vs 0.66, respectively). […] The median OS was 16 months and the 1-, 3-, and 5-year survival rates were 55.2%, 33.7%, and 26.4%, respectively. […] In general, patients with local and stage G1 disease have better survival rates. Overall, patients benefited from surgery (HR, 1.73; 95% CI: 1.5971.874; P.05). […] The nomogram showed a good prediction accuracy. […] The C-index of the nomogram for OS was 0.74 (95% CI: 0.730.75). […] The C-index of our nomogram was significantly better than those of the 6th and 7th editions of the AJCC staging system, indicating the superiority of our nomogram.
  • #13
    https://journals.lww.com/md-journal/fulltext/2025/01030/trends_in_the_incidence,_survival,_and_prognostic.53.aspx
    This study aimed to investigate the epidemiological trends of angiosarcoma and to establish a tool to estimate its prognosis. […] The median survival time is approximately 30 to 50 months, with a 5-year survival rate of 10% to 50%. […] In multivariable analyses, age, sex, marital status, grade, historical stage, surgery, site, and tumor size were independent prognostic factors for angiosarcoma. […] The concordance index of the nomogram was significantly higher than that of the American Joint Committee on Cancer (AJCC) 6th edition and the AJCC 7th edition (0.74 vs 0.61 vs 0.66, respectively). […] The median OS was 16 months and the 1-, 3-, and 5-year survival rates were 55.2%, 33.7%, and 26.4%, respectively. […] In general, patients with local and stage G1 disease have better survival rates. Overall, patients benefited from surgery (HR, 1.73; 95% CI: 1.5971.874; P.05). […] The nomogram showed a good prediction accuracy. […] The C-index of the nomogram for OS was 0.74 (95% CI: 0.730.75). […] The C-index of our nomogram was significantly better than those of the 6th and 7th editions of the AJCC staging system, indicating the superiority of our nomogram.
  • #14 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    Angiosarcoma is a rare soft tissue sarcoma, and the data about its clinicopathological features and prognostic factors are limited. […] Of the 113 patients with followup data, 46 patients succumbed to the disease with a median interval of 10 months. Tumor recurrence/metastasis was identified in 66 patients with a median interval of 4 months. The disease-free survival (DFS) rate at 5 years was 19.3% and the overall survival (OS) rate at 5 years was 40.8%. […] The prognosis for angiosarcoma is also associated with the primary site of the tumor. Fayette et al retrospectively analyzed the clinicopathological features of 161 cases of angiosarcoma, and demonstrated that the patients’ survival was significantly impacted by the primary site. A primary site in the liver or heart was associated with a poor prognosis, whereas primary soft tissue tumors were associated with a relatively improved prognosis.
  • #15 Survival predictors of metastatic angiosarcomas: a surveillance, epidemiology, and end results program population-based retrospective study | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-020-07300-7
    Angiosarcomas (AS) have poor prognosis and often metastasize to distant sites. The potential predictors of metastatic angiosarcomas (MAS) have not been extensively investigated. The main objective of this study was to identify survival predictors of MAS. […] Overall, 96 patients (33.8%) had two or more metastatic sites. The 1- and 3-year overall survival (OS) rates were 20.8 and 3.8% while 1- and 3-year cancer-specific survival (CSS) rates were 22.0 and 5.2%, respectively. […] MAS is associated with extremely poor survival. Chemotherapy, RT, and tumor size are independent predictors of OS. Chemotherapy and tumor size are independent prognostic factors of CSS. […] In this study, we analyzed the survival predictors of MAS, known for its extremely poor survival rates, in 284 patients. Chemotherapy, RT and tumor size 10cm were identified as independent protective predictors of OS. Chemotherapy and tumor size 10cm were independent favorable prognostic factors of CSS. Grade IV was associated with poorer survival of CSS. The number of metastatic sites did not appear to affect OS and CSS. Based on the collective findings, we recommend chemotherapy as the preferred treatment option for MAS patients.
  • #16 Survival predictors of metastatic angiosarcomas: a surveillance, epidemiology, and end results program population-based retrospective study | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-020-07300-7
    Angiosarcomas (AS) have poor prognosis and often metastasize to distant sites. The potential predictors of metastatic angiosarcomas (MAS) have not been extensively investigated. The main objective of this study was to identify survival predictors of MAS. […] Overall, 96 patients (33.8%) had two or more metastatic sites. The 1- and 3-year overall survival (OS) rates were 20.8 and 3.8% while 1- and 3-year cancer-specific survival (CSS) rates were 22.0 and 5.2%, respectively. […] MAS is associated with extremely poor survival. Chemotherapy, RT, and tumor size are independent predictors of OS. Chemotherapy and tumor size are independent prognostic factors of CSS. […] In this study, we analyzed the survival predictors of MAS, known for its extremely poor survival rates, in 284 patients. Chemotherapy, RT and tumor size 10cm were identified as independent protective predictors of OS. Chemotherapy and tumor size 10cm were independent favorable prognostic factors of CSS. Grade IV was associated with poorer survival of CSS. The number of metastatic sites did not appear to affect OS and CSS. Based on the collective findings, we recommend chemotherapy as the preferred treatment option for MAS patients.
  • #17 Survival predictors of metastatic angiosarcomas: a surveillance, epidemiology, and end results program population-based retrospective study | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-020-07300-7
    Angiosarcomas (AS) have poor prognosis and often metastasize to distant sites. The potential predictors of metastatic angiosarcomas (MAS) have not been extensively investigated. The main objective of this study was to identify survival predictors of MAS. […] Overall, 96 patients (33.8%) had two or more metastatic sites. The 1- and 3-year overall survival (OS) rates were 20.8 and 3.8% while 1- and 3-year cancer-specific survival (CSS) rates were 22.0 and 5.2%, respectively. […] MAS is associated with extremely poor survival. Chemotherapy, RT, and tumor size are independent predictors of OS. Chemotherapy and tumor size are independent prognostic factors of CSS. […] In this study, we analyzed the survival predictors of MAS, known for its extremely poor survival rates, in 284 patients. Chemotherapy, RT and tumor size 10cm were identified as independent protective predictors of OS. Chemotherapy and tumor size 10cm were independent favorable prognostic factors of CSS. Grade IV was associated with poorer survival of CSS. The number of metastatic sites did not appear to affect OS and CSS. Based on the collective findings, we recommend chemotherapy as the preferred treatment option for MAS patients.
  • #18 Survival predictors of metastatic angiosarcomas: a surveillance, epidemiology, and end results program population-based retrospective study | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-020-07300-7
    Angiosarcomas (AS) have poor prognosis and often metastasize to distant sites. The potential predictors of metastatic angiosarcomas (MAS) have not been extensively investigated. The main objective of this study was to identify survival predictors of MAS. […] Overall, 96 patients (33.8%) had two or more metastatic sites. The 1- and 3-year overall survival (OS) rates were 20.8 and 3.8% while 1- and 3-year cancer-specific survival (CSS) rates were 22.0 and 5.2%, respectively. […] MAS is associated with extremely poor survival. Chemotherapy, RT, and tumor size are independent predictors of OS. Chemotherapy and tumor size are independent prognostic factors of CSS. […] In this study, we analyzed the survival predictors of MAS, known for its extremely poor survival rates, in 284 patients. Chemotherapy, RT and tumor size 10cm were identified as independent protective predictors of OS. Chemotherapy and tumor size 10cm were independent favorable prognostic factors of CSS. Grade IV was associated with poorer survival of CSS. The number of metastatic sites did not appear to affect OS and CSS. Based on the collective findings, we recommend chemotherapy as the preferred treatment option for MAS patients.
  • #19 Survival predictors of metastatic angiosarcomas: a surveillance, epidemiology, and end results program population-based retrospective study | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-020-07300-7
    Angiosarcomas (AS) have poor prognosis and often metastasize to distant sites. The potential predictors of metastatic angiosarcomas (MAS) have not been extensively investigated. The main objective of this study was to identify survival predictors of MAS. […] Overall, 96 patients (33.8%) had two or more metastatic sites. The 1- and 3-year overall survival (OS) rates were 20.8 and 3.8% while 1- and 3-year cancer-specific survival (CSS) rates were 22.0 and 5.2%, respectively. […] MAS is associated with extremely poor survival. Chemotherapy, RT, and tumor size are independent predictors of OS. Chemotherapy and tumor size are independent prognostic factors of CSS. […] In this study, we analyzed the survival predictors of MAS, known for its extremely poor survival rates, in 284 patients. Chemotherapy, RT and tumor size 10cm were identified as independent protective predictors of OS. Chemotherapy and tumor size 10cm were independent favorable prognostic factors of CSS. Grade IV was associated with poorer survival of CSS. The number of metastatic sites did not appear to affect OS and CSS. Based on the collective findings, we recommend chemotherapy as the preferred treatment option for MAS patients.
  • #20 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #21 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    Angiosarcoma is a rare soft tissue sarcoma, and the data about its clinicopathological features and prognostic factors are limited. […] Of the 113 patients with followup data, 46 patients succumbed to the disease with a median interval of 10 months. Tumor recurrence/metastasis was identified in 66 patients with a median interval of 4 months. The disease-free survival (DFS) rate at 5 years was 19.3% and the overall survival (OS) rate at 5 years was 40.8%. […] The prognosis for angiosarcoma is also associated with the primary site of the tumor. Fayette et al retrospectively analyzed the clinicopathological features of 161 cases of angiosarcoma, and demonstrated that the patients’ survival was significantly impacted by the primary site. A primary site in the liver or heart was associated with a poor prognosis, whereas primary soft tissue tumors were associated with a relatively improved prognosis.
  • #22 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    The results revealed in the present study suggest that angiosarcoma is a family of sarcomas with distinctive behavior depending on the primary site. According to the primary site, all the cases in the present study were divided into four groups: i) Head and neck; ii) breast; iii) viscera (including internal organ and bone); and iv) soft tissue (including trunk and extremities). Overall, angiosarcomas exhibited a similar distribution between sexes (female/male, 103/97; ratio, 1.1:1). […] The results of the present study also demonstrated that the primary sites of the tumor affect the prognosis, which may be used to classify angiosarcoma. DFS and OS were affected by the primary site of angiosarcoma in the present study. The head and neck angiosarcomas were associated with the poorest survival, with a 5-year OS of 28.0%. However, the outcomes of patients with breast angiosarcomas in the present study were favorable, with a 5-year OS of 87.5%.
  • #23 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #24 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #25 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/276512-overview
    In a study of 346 primary hepatic angiosarcoma (PHA) patients, the median survival was 1.9 months. Those who underwent surgical resection had a higher median survival rate of 7.7 months. Chemotherapy treatment also increased median survival to 5.1 months. […] More than 50% of patients develop metastatic disease, with the lung the primary organ involved. […] Angiosarcoma of the soft tissue is a high-grade sarcoma with a high rate of death and short survival time. A large number of patients, 50% in some series, also had metastasis, and a significant number (20%) had local recurrences. […] Older patient age, retroperitoneum location, and larger tumor size are unfavorable prognostic factors. Approximately 66% of retroperitoneal angiosarcomas recur locally in the tumor bed and can recur diffusely throughout the peritoneal cavity (angiosarcomatosis).
  • #26 Primary splenic angiosarcoma: a case series of a rare oncological entity and diagnostic challenge
    https://medicaljournalssweden.se/actaoncologica/article/download/35412/45905?inline=1
    Primary angiosarcoma of the spleen (PAS), an exceptionally rare and aggressive neoplasm with high metastatic risk (70%-85%), is frequently diagnosed in an advanced or metastatic stage. […] Unfortunately, the prognosis is dismal with a median overall survival (OS) of only 5-6 months. In case of early detection, splenectomy has been reported to increase the median OS to 14 months. […] While it is crucial to acknowledge that further trial data is required to evaluate the efficacy of emerging treatment regimens, designing and conducting trials for PAS is challenging given its scarcity and aggressive behavior. […] When diagnosed early, the recommended primary treatment for PAS is splenectomy before any potential splenic rupture occurs, as it is the only treatment that benefits OS. It should be noted however, that splenectomy is rarely curative.
  • #27 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    The results revealed in the present study suggest that angiosarcoma is a family of sarcomas with distinctive behavior depending on the primary site. According to the primary site, all the cases in the present study were divided into four groups: i) Head and neck; ii) breast; iii) viscera (including internal organ and bone); and iv) soft tissue (including trunk and extremities). Overall, angiosarcomas exhibited a similar distribution between sexes (female/male, 103/97; ratio, 1.1:1). […] The results of the present study also demonstrated that the primary sites of the tumor affect the prognosis, which may be used to classify angiosarcoma. DFS and OS were affected by the primary site of angiosarcoma in the present study. The head and neck angiosarcomas were associated with the poorest survival, with a 5-year OS of 28.0%. However, the outcomes of patients with breast angiosarcomas in the present study were favorable, with a 5-year OS of 87.5%.
  • #28 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    The results revealed in the present study suggest that angiosarcoma is a family of sarcomas with distinctive behavior depending on the primary site. According to the primary site, all the cases in the present study were divided into four groups: i) Head and neck; ii) breast; iii) viscera (including internal organ and bone); and iv) soft tissue (including trunk and extremities). Overall, angiosarcomas exhibited a similar distribution between sexes (female/male, 103/97; ratio, 1.1:1). […] The results of the present study also demonstrated that the primary sites of the tumor affect the prognosis, which may be used to classify angiosarcoma. DFS and OS were affected by the primary site of angiosarcoma in the present study. The head and neck angiosarcomas were associated with the poorest survival, with a 5-year OS of 28.0%. However, the outcomes of patients with breast angiosarcomas in the present study were favorable, with a 5-year OS of 87.5%.
  • #29 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #30 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Similarly, primary tumor size influenced survival; median OS of patients with tumors 5 cm and 5 cm were 60 months and 10 months, respectively. […] From a histologic perspective, the presence of necrosis was associated with worse outcomes (median OS of 9 months for tumors with necrosis vs. 60 months for tumors without necrosis, P = 0.003). […] Surgery was clearly advantageous when either the entire cohort or patients presenting with localized disease were considered. […] Chemotherapy appears to benefit patients with metastatic disease. […] The overall poor outcomes following currently available interventions, particularly for patients presenting with metastatic disease, necessitate an urgent search for novel therapies.
  • #31 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    Tumor size and tumor differentiation are important prognostic factors in soft tissue sarcoma, and their value in angiosarcoma may also be confirmed in the present study. The present univariate analysis confirmed that tumor size and histological grade are prognostic factors for DFS and OS. Tumors size 5 cm and high-grade histological differentiation predicted poor prognosis, as previously described. […] The present study identified that tumor differentiation and recurrence/metastasis were independent prognostic factors using multivariate survival analysis. Although certain parameters may not be independent prognostic factors, the present study hypothesizes that all the aforementioned factors mentioned should be considered by physicians when assessing the biological behavior of the tumor and choosing the therapeutic strategies.
  • #32 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #33 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Similarly, primary tumor size influenced survival; median OS of patients with tumors 5 cm and 5 cm were 60 months and 10 months, respectively. […] From a histologic perspective, the presence of necrosis was associated with worse outcomes (median OS of 9 months for tumors with necrosis vs. 60 months for tumors without necrosis, P = 0.003). […] Surgery was clearly advantageous when either the entire cohort or patients presenting with localized disease were considered. […] Chemotherapy appears to benefit patients with metastatic disease. […] The overall poor outcomes following currently available interventions, particularly for patients presenting with metastatic disease, necessitate an urgent search for novel therapies.
  • #34 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Similarly, primary tumor size influenced survival; median OS of patients with tumors 5 cm and 5 cm were 60 months and 10 months, respectively. […] From a histologic perspective, the presence of necrosis was associated with worse outcomes (median OS of 9 months for tumors with necrosis vs. 60 months for tumors without necrosis, P = 0.003). […] Surgery was clearly advantageous when either the entire cohort or patients presenting with localized disease were considered. […] Chemotherapy appears to benefit patients with metastatic disease. […] The overall poor outcomes following currently available interventions, particularly for patients presenting with metastatic disease, necessitate an urgent search for novel therapies.
  • #35 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    Tumor size and tumor differentiation are important prognostic factors in soft tissue sarcoma, and their value in angiosarcoma may also be confirmed in the present study. The present univariate analysis confirmed that tumor size and histological grade are prognostic factors for DFS and OS. Tumors size 5 cm and high-grade histological differentiation predicted poor prognosis, as previously described. […] The present study identified that tumor differentiation and recurrence/metastasis were independent prognostic factors using multivariate survival analysis. Although certain parameters may not be independent prognostic factors, the present study hypothesizes that all the aforementioned factors mentioned should be considered by physicians when assessing the biological behavior of the tumor and choosing the therapeutic strategies.
  • #36 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/276512-overview
    High-grade angiosarcomas exhibit extremely aggressive behavior with rapid local growth and early disseminated metastasis. Prognosis depends on the histologic grade, with the disease-free survival rate reported as 95% in grade 1 tumors, 62% in grade 2, and 20% in grade 3. Multicentricity does not affect prognosis. […] Despite aggressive treatment, prognosis is poor. The median survival ranges from 15-24 months, with a 5-year survival rate of 12-33%. Local failure and metastases to local cervical lymph nodes are common. […] Unlike other sarcomas, grade is not useful in predicting survival. No correlation exists between appearance (eg, ulcerated, nodular, diffuse) and survival or local recurrence. […] Findings of significantly favorable prognostic importance appear to be smaller tumor size ( 5 cm), complete surgical resection, and a moderate or marked lymphoid infiltrate in and around the tumor. […] Unresectable lesions and metastatic disease at diagnosis suggest a dismal prognosis. Death can occur either from local extension or metastasis. Delayed diagnosis and treatment explain, in part, the poor prognosis of cutaneous angiosarcomas.
  • #37 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Similarly, primary tumor size influenced survival; median OS of patients with tumors 5 cm and 5 cm were 60 months and 10 months, respectively. […] From a histologic perspective, the presence of necrosis was associated with worse outcomes (median OS of 9 months for tumors with necrosis vs. 60 months for tumors without necrosis, P = 0.003). […] Surgery was clearly advantageous when either the entire cohort or patients presenting with localized disease were considered. […] Chemotherapy appears to benefit patients with metastatic disease. […] The overall poor outcomes following currently available interventions, particularly for patients presenting with metastatic disease, necessitate an urgent search for novel therapies.
  • #38 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Similarly, primary tumor size influenced survival; median OS of patients with tumors 5 cm and 5 cm were 60 months and 10 months, respectively. […] From a histologic perspective, the presence of necrosis was associated with worse outcomes (median OS of 9 months for tumors with necrosis vs. 60 months for tumors without necrosis, P = 0.003). […] Surgery was clearly advantageous when either the entire cohort or patients presenting with localized disease were considered. […] Chemotherapy appears to benefit patients with metastatic disease. […] The overall poor outcomes following currently available interventions, particularly for patients presenting with metastatic disease, necessitate an urgent search for novel therapies.
  • #39 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/276512-overview
    High-grade angiosarcomas exhibit extremely aggressive behavior with rapid local growth and early disseminated metastasis. Prognosis depends on the histologic grade, with the disease-free survival rate reported as 95% in grade 1 tumors, 62% in grade 2, and 20% in grade 3. Multicentricity does not affect prognosis. […] Despite aggressive treatment, prognosis is poor. The median survival ranges from 15-24 months, with a 5-year survival rate of 12-33%. Local failure and metastases to local cervical lymph nodes are common. […] Unlike other sarcomas, grade is not useful in predicting survival. No correlation exists between appearance (eg, ulcerated, nodular, diffuse) and survival or local recurrence. […] Findings of significantly favorable prognostic importance appear to be smaller tumor size ( 5 cm), complete surgical resection, and a moderate or marked lymphoid infiltrate in and around the tumor. […] Unresectable lesions and metastatic disease at diagnosis suggest a dismal prognosis. Death can occur either from local extension or metastasis. Delayed diagnosis and treatment explain, in part, the poor prognosis of cutaneous angiosarcomas.
  • #40 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #41 Angiosarcoma: clinical outcomes and prognostic factors, a single-center analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC11196347/
    This study sought to investigate oncological outcomes and prognostic factors for patients with angiosarcomas (AS). […] Overall survival (OS), local control (LC) and local progression-free survival (LPFS) were assessed by Kaplan-Meier estimator. […] Multivariable Cox regression of OS showed a significant association with age per year (hazard ratio (HR): 1.03, p=0.044) and metastatic disease at presentation (hazard ratio: 3.24, p=0.015). For LPFS, age per year (HR: 1.04, p=0.008), locally recurrent disease at presentation (HR: 5.32, p=0.013), and metastatic disease at presentation (HR: 4.06, p=0.009) had significant associations. […] Older age and metastatic disease at initial presentation status were negatively associated with OS and LPFS. […] Despite intensive treatment, the overall prognosis of patients is limited. Age and metastatic disease at presentation were significant adverse prognostic factors for OS, and local disease progression after therapy.
  • #42 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #43 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #44 Angiosarcoma: clinical outcomes and prognostic factors, a single-center analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC11196347/
    This study sought to investigate oncological outcomes and prognostic factors for patients with angiosarcomas (AS). […] Overall survival (OS), local control (LC) and local progression-free survival (LPFS) were assessed by Kaplan-Meier estimator. […] Multivariable Cox regression of OS showed a significant association with age per year (hazard ratio (HR): 1.03, p=0.044) and metastatic disease at presentation (hazard ratio: 3.24, p=0.015). For LPFS, age per year (HR: 1.04, p=0.008), locally recurrent disease at presentation (HR: 5.32, p=0.013), and metastatic disease at presentation (HR: 4.06, p=0.009) had significant associations. […] Older age and metastatic disease at initial presentation status were negatively associated with OS and LPFS. […] Despite intensive treatment, the overall prognosis of patients is limited. Age and metastatic disease at presentation were significant adverse prognostic factors for OS, and local disease progression after therapy.
  • #45 Prognostic and predictive factors for angiosarcoma patients receiving paclitaxel once weekly plus or minus bevacizumab: an ancillary study derived from a randomized clinical trial | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4828-1
    Among the 51 patients enrolled and treated in this trial, biomarker analysis was performed for 42: 18 in Arm A (single-agent) and 24 in Arm B (combination). […] According to univariate analysis, factors associated with a poor PFS were as follows: visceral angiosarcoma, de novo angiosarcoma, and high PlGF and low VEGF-C baseline values. In multivariate analysis, de novo angiosarcoma (HR=2.5; p=0.024) and baseline VEGF-C value (HR=0.7; p=0.003) were significant prognostic factors. […] De novo angiosarcoma and a low baseline level of VEGF-C were found to be associated with a poor prognosis. […] The results of the present study confirmed that adding bevacizumab to weekly paclitaxel did not improve the PFS of advanced angiosarcoma. […] At baseline, high values of circulating PlGF and low values of VEGF-C were associated with poor outcomes.
  • #46 Prognostic and predictive factors for angiosarcoma patients receiving paclitaxel once weekly plus or minus bevacizumab: an ancillary study derived from a randomized clinical trial | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4828-1
    Finally, an increase in FGF between day 1 and day 8 was associated with a heightened risk of disease progression. […] When treated with weekly paclitaxel, the outcome of angiosarcoma patients in advanced stages is better for radiation-induced tumors than it is for de novo angiosarcoma. […] A low level of circulating VEGF-C and a high level of circulating PlGF at baseline were found to be associated with a poor outcome.
  • #47 Prognostic and predictive factors for angiosarcoma patients receiving paclitaxel once weekly plus or minus bevacizumab: an ancillary study derived from a randomized clinical trial | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4828-1
    Among the 51 patients enrolled and treated in this trial, biomarker analysis was performed for 42: 18 in Arm A (single-agent) and 24 in Arm B (combination). […] According to univariate analysis, factors associated with a poor PFS were as follows: visceral angiosarcoma, de novo angiosarcoma, and high PlGF and low VEGF-C baseline values. In multivariate analysis, de novo angiosarcoma (HR=2.5; p=0.024) and baseline VEGF-C value (HR=0.7; p=0.003) were significant prognostic factors. […] De novo angiosarcoma and a low baseline level of VEGF-C were found to be associated with a poor prognosis. […] The results of the present study confirmed that adding bevacizumab to weekly paclitaxel did not improve the PFS of advanced angiosarcoma. […] At baseline, high values of circulating PlGF and low values of VEGF-C were associated with poor outcomes.
  • #48 Prognostic and predictive factors for angiosarcoma patients receiving paclitaxel once weekly plus or minus bevacizumab: an ancillary study derived from a randomized clinical trial | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4828-1
    Among the 51 patients enrolled and treated in this trial, biomarker analysis was performed for 42: 18 in Arm A (single-agent) and 24 in Arm B (combination). […] According to univariate analysis, factors associated with a poor PFS were as follows: visceral angiosarcoma, de novo angiosarcoma, and high PlGF and low VEGF-C baseline values. In multivariate analysis, de novo angiosarcoma (HR=2.5; p=0.024) and baseline VEGF-C value (HR=0.7; p=0.003) were significant prognostic factors. […] De novo angiosarcoma and a low baseline level of VEGF-C were found to be associated with a poor prognosis. […] The results of the present study confirmed that adding bevacizumab to weekly paclitaxel did not improve the PFS of advanced angiosarcoma. […] At baseline, high values of circulating PlGF and low values of VEGF-C were associated with poor outcomes.
  • #49 Prognostic and predictive factors for angiosarcoma patients receiving paclitaxel once weekly plus or minus bevacizumab: an ancillary study derived from a randomized clinical trial | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4828-1
    Finally, an increase in FGF between day 1 and day 8 was associated with a heightened risk of disease progression. […] When treated with weekly paclitaxel, the outcome of angiosarcoma patients in advanced stages is better for radiation-induced tumors than it is for de novo angiosarcoma. […] A low level of circulating VEGF-C and a high level of circulating PlGF at baseline were found to be associated with a poor outcome.
  • #50 Prognostic and predictive factors for angiosarcoma patients receiving paclitaxel once weekly plus or minus bevacizumab: an ancillary study derived from a randomized clinical trial | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4828-1
    Finally, an increase in FGF between day 1 and day 8 was associated with a heightened risk of disease progression. […] When treated with weekly paclitaxel, the outcome of angiosarcoma patients in advanced stages is better for radiation-induced tumors than it is for de novo angiosarcoma. […] A low level of circulating VEGF-C and a high level of circulating PlGF at baseline were found to be associated with a poor outcome.
  • #51 Angiosarcoma: clinical outcomes and prognostic factors, a single-center analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC11196347/
    This study sought to investigate oncological outcomes and prognostic factors for patients with angiosarcomas (AS). […] Overall survival (OS), local control (LC) and local progression-free survival (LPFS) were assessed by Kaplan-Meier estimator. […] Multivariable Cox regression of OS showed a significant association with age per year (hazard ratio (HR): 1.03, p=0.044) and metastatic disease at presentation (hazard ratio: 3.24, p=0.015). For LPFS, age per year (HR: 1.04, p=0.008), locally recurrent disease at presentation (HR: 5.32, p=0.013), and metastatic disease at presentation (HR: 4.06, p=0.009) had significant associations. […] Older age and metastatic disease at initial presentation status were negatively associated with OS and LPFS. […] Despite intensive treatment, the overall prognosis of patients is limited. Age and metastatic disease at presentation were significant adverse prognostic factors for OS, and local disease progression after therapy.
  • #52
    https://journals.lww.com/md-journal/fulltext/2025/01030/trends_in_the_incidence,_survival,_and_prognostic.53.aspx
    This study aimed to investigate the epidemiological trends of angiosarcoma and to establish a tool to estimate its prognosis. […] The median survival time is approximately 30 to 50 months, with a 5-year survival rate of 10% to 50%. […] In multivariable analyses, age, sex, marital status, grade, historical stage, surgery, site, and tumor size were independent prognostic factors for angiosarcoma. […] The concordance index of the nomogram was significantly higher than that of the American Joint Committee on Cancer (AJCC) 6th edition and the AJCC 7th edition (0.74 vs 0.61 vs 0.66, respectively). […] The median OS was 16 months and the 1-, 3-, and 5-year survival rates were 55.2%, 33.7%, and 26.4%, respectively. […] In general, patients with local and stage G1 disease have better survival rates. Overall, patients benefited from surgery (HR, 1.73; 95% CI: 1.5971.874; P.05). […] The nomogram showed a good prediction accuracy. […] The C-index of the nomogram for OS was 0.74 (95% CI: 0.730.75). […] The C-index of our nomogram was significantly better than those of the 6th and 7th editions of the AJCC staging system, indicating the superiority of our nomogram.
  • #53 Prognostic nomograms for predicting overall survival and cancer-specific survival in patients with angiosarcoma, a SEER population-based study | Scientific Reports
    https://www.nature.com/articles/s41598-022-07444-5
    It has been reported that the median overall survival (OS) varied from 30 to 50 months for AS patients, resulting in a poor prognosis. […] Based on the univariate and multivariate regression, gender, age, AJCC stage group 7th ed, T and N stage 7th ed, histologic grade and primary site were recognized as the independent predictors of prognosis which were utilized to establish nomograms. […] The C-index of the model in the internal and external validation were all greater than 0.7 for OS (0.757, 0.749) and CSS (0.762, 0.756). […] This nomogram provides important clinical use in AS which are listed as follows. Firstly, Clinicians can more accurately and faster to estimate the survival chances and recognize the personalized risk of AS patients so as to adopt corresponding interventions. […] Taken together, our study constructed a novel predictive model to identify the risk in post-operative AS patients with no metastasis. The nomograms were capable to predict 3- and 5-year OS and CSS.
  • #54 Angiosarcoma: clinical outcomes and prognostic factors, a single-center analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC11196347/
    This study sought to investigate oncological outcomes and prognostic factors for patients with angiosarcomas (AS). […] Overall survival (OS), local control (LC) and local progression-free survival (LPFS) were assessed by Kaplan-Meier estimator. […] Multivariable Cox regression of OS showed a significant association with age per year (hazard ratio (HR): 1.03, p=0.044) and metastatic disease at presentation (hazard ratio: 3.24, p=0.015). For LPFS, age per year (HR: 1.04, p=0.008), locally recurrent disease at presentation (HR: 5.32, p=0.013), and metastatic disease at presentation (HR: 4.06, p=0.009) had significant associations. […] Older age and metastatic disease at initial presentation status were negatively associated with OS and LPFS. […] Despite intensive treatment, the overall prognosis of patients is limited. Age and metastatic disease at presentation were significant adverse prognostic factors for OS, and local disease progression after therapy.
  • #55 Prognostic nomograms for predicting overall survival and cancer-specific survival in patients with angiosarcoma, a SEER population-based study | Scientific Reports
    https://www.nature.com/articles/s41598-022-07444-5
    It has been reported that the median overall survival (OS) varied from 30 to 50 months for AS patients, resulting in a poor prognosis. […] Based on the univariate and multivariate regression, gender, age, AJCC stage group 7th ed, T and N stage 7th ed, histologic grade and primary site were recognized as the independent predictors of prognosis which were utilized to establish nomograms. […] The C-index of the model in the internal and external validation were all greater than 0.7 for OS (0.757, 0.749) and CSS (0.762, 0.756). […] This nomogram provides important clinical use in AS which are listed as follows. Firstly, Clinicians can more accurately and faster to estimate the survival chances and recognize the personalized risk of AS patients so as to adopt corresponding interventions. […] Taken together, our study constructed a novel predictive model to identify the risk in post-operative AS patients with no metastasis. The nomograms were capable to predict 3- and 5-year OS and CSS.
  • #56
    https://journals.lww.com/md-journal/fulltext/2025/01030/trends_in_the_incidence,_survival,_and_prognostic.53.aspx
    This study aimed to investigate the epidemiological trends of angiosarcoma and to establish a tool to estimate its prognosis. […] The median survival time is approximately 30 to 50 months, with a 5-year survival rate of 10% to 50%. […] In multivariable analyses, age, sex, marital status, grade, historical stage, surgery, site, and tumor size were independent prognostic factors for angiosarcoma. […] The concordance index of the nomogram was significantly higher than that of the American Joint Committee on Cancer (AJCC) 6th edition and the AJCC 7th edition (0.74 vs 0.61 vs 0.66, respectively). […] The median OS was 16 months and the 1-, 3-, and 5-year survival rates were 55.2%, 33.7%, and 26.4%, respectively. […] In general, patients with local and stage G1 disease have better survival rates. Overall, patients benefited from surgery (HR, 1.73; 95% CI: 1.5971.874; P.05). […] The nomogram showed a good prediction accuracy. […] The C-index of the nomogram for OS was 0.74 (95% CI: 0.730.75). […] The C-index of our nomogram was significantly better than those of the 6th and 7th editions of the AJCC staging system, indicating the superiority of our nomogram.
  • #57 Real-World Clinical Outcomes and Prognostic Factors for Patients with Advanced Angiosarcoma who Received Systemic Treatment
    https://e-crt.org/journal/view.php?number=3219
    Angiosarcoma is a highly aggressive mesenchymal tumor. Although systemic chemotherapy is often considered for the inoperable or metastatic angiosarcoma, the outcome of such treatment is unsatisfactory and poorly delineated. […] The prognosis of angiosarcoma is generally poor even in localized disease with 5-year overall survival (OS) up to only 60% with a median survival of around 310 months for metastatic disease. […] Patients with primary hepatic angiosarcoma tended to have poorer OS than those without liver involvement. […] Presence of liver involvement was significantly associated with poor OS. […] In the univariate Cox proportional hazard regression analysis, ECOG PS and presence of liver involvement were significantly associated with poor OS. […] In addition, higher pre-chemotherapy bilirubin and lower pre-chemotherapy albumin were associated with poor OS. […] Treatment with first-line wPac and later-line pazopanib seems to provide survival benefit, especially for patients with advanced angiosarcoma without liver involvement.
  • #58 Real-World Clinical Outcomes and Prognostic Factors for Patients with Advanced Angiosarcoma who Received Systemic Treatment
    https://e-crt.org/journal/view.php?number=3219
    Angiosarcoma is a highly aggressive mesenchymal tumor. Although systemic chemotherapy is often considered for the inoperable or metastatic angiosarcoma, the outcome of such treatment is unsatisfactory and poorly delineated. […] The prognosis of angiosarcoma is generally poor even in localized disease with 5-year overall survival (OS) up to only 60% with a median survival of around 310 months for metastatic disease. […] Patients with primary hepatic angiosarcoma tended to have poorer OS than those without liver involvement. […] Presence of liver involvement was significantly associated with poor OS. […] In the univariate Cox proportional hazard regression analysis, ECOG PS and presence of liver involvement were significantly associated with poor OS. […] In addition, higher pre-chemotherapy bilirubin and lower pre-chemotherapy albumin were associated with poor OS. […] Treatment with first-line wPac and later-line pazopanib seems to provide survival benefit, especially for patients with advanced angiosarcoma without liver involvement.
  • #59 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Similarly, primary tumor size influenced survival; median OS of patients with tumors 5 cm and 5 cm were 60 months and 10 months, respectively. […] From a histologic perspective, the presence of necrosis was associated with worse outcomes (median OS of 9 months for tumors with necrosis vs. 60 months for tumors without necrosis, P = 0.003). […] Surgery was clearly advantageous when either the entire cohort or patients presenting with localized disease were considered. […] Chemotherapy appears to benefit patients with metastatic disease. […] The overall poor outcomes following currently available interventions, particularly for patients presenting with metastatic disease, necessitate an urgent search for novel therapies.
  • #60 Survival predictors of metastatic angiosarcomas: a surveillance, epidemiology, and end results program population-based retrospective study | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-020-07300-7
    Angiosarcomas (AS) have poor prognosis and often metastasize to distant sites. The potential predictors of metastatic angiosarcomas (MAS) have not been extensively investigated. The main objective of this study was to identify survival predictors of MAS. […] Overall, 96 patients (33.8%) had two or more metastatic sites. The 1- and 3-year overall survival (OS) rates were 20.8 and 3.8% while 1- and 3-year cancer-specific survival (CSS) rates were 22.0 and 5.2%, respectively. […] MAS is associated with extremely poor survival. Chemotherapy, RT, and tumor size are independent predictors of OS. Chemotherapy and tumor size are independent prognostic factors of CSS. […] In this study, we analyzed the survival predictors of MAS, known for its extremely poor survival rates, in 284 patients. Chemotherapy, RT and tumor size 10cm were identified as independent protective predictors of OS. Chemotherapy and tumor size 10cm were independent favorable prognostic factors of CSS. Grade IV was associated with poorer survival of CSS. The number of metastatic sites did not appear to affect OS and CSS. Based on the collective findings, we recommend chemotherapy as the preferred treatment option for MAS patients.
  • #61 Real-World Clinical Outcomes and Prognostic Factors for Patients with Advanced Angiosarcoma who Received Systemic Treatment
    https://e-crt.org/journal/view.php?number=3219
    Angiosarcoma is a highly aggressive mesenchymal tumor. Although systemic chemotherapy is often considered for the inoperable or metastatic angiosarcoma, the outcome of such treatment is unsatisfactory and poorly delineated. […] The prognosis of angiosarcoma is generally poor even in localized disease with 5-year overall survival (OS) up to only 60% with a median survival of around 310 months for metastatic disease. […] Patients with primary hepatic angiosarcoma tended to have poorer OS than those without liver involvement. […] Presence of liver involvement was significantly associated with poor OS. […] In the univariate Cox proportional hazard regression analysis, ECOG PS and presence of liver involvement were significantly associated with poor OS. […] In addition, higher pre-chemotherapy bilirubin and lower pre-chemotherapy albumin were associated with poor OS. […] Treatment with first-line wPac and later-line pazopanib seems to provide survival benefit, especially for patients with advanced angiosarcoma without liver involvement.
  • #62 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Similarly, primary tumor size influenced survival; median OS of patients with tumors 5 cm and 5 cm were 60 months and 10 months, respectively. […] From a histologic perspective, the presence of necrosis was associated with worse outcomes (median OS of 9 months for tumors with necrosis vs. 60 months for tumors without necrosis, P = 0.003). […] Surgery was clearly advantageous when either the entire cohort or patients presenting with localized disease were considered. […] Chemotherapy appears to benefit patients with metastatic disease. […] The overall poor outcomes following currently available interventions, particularly for patients presenting with metastatic disease, necessitate an urgent search for novel therapies.
  • #63 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/276512-overview
    All angiosarcomas tend to be aggressive and are often multicentric. These tumors have high rates of local recurrence and metastasis because of their intrinsic biologic properties and because they are often misdiagnosed, leading to a poor prognosis and a high mortality rate. Malignant vascular tumors are clinically aggressive, difficult to treat, and have a reported 5-year survival rate around 20-35%. […] Advanced stage at presentation and lack of radical or wide excision are associated with higher recurrence rates, higher distant metastasis rates, and worsened survival. This is in contrast to the 5-year survival for all types of soft tissue sarcomas, which is around 65%. […] Angiosarcoma of the viscera (particularly liver and heart) and retroperitoneal disease are especially associated with poor outcome.
  • #64 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Similarly, primary tumor size influenced survival; median OS of patients with tumors 5 cm and 5 cm were 60 months and 10 months, respectively. […] From a histologic perspective, the presence of necrosis was associated with worse outcomes (median OS of 9 months for tumors with necrosis vs. 60 months for tumors without necrosis, P = 0.003). […] Surgery was clearly advantageous when either the entire cohort or patients presenting with localized disease were considered. […] Chemotherapy appears to benefit patients with metastatic disease. […] The overall poor outcomes following currently available interventions, particularly for patients presenting with metastatic disease, necessitate an urgent search for novel therapies.
  • #65 Real-World Clinical Outcomes and Prognostic Factors for Patients with Advanced Angiosarcoma who Received Systemic Treatment
    https://e-crt.org/journal/view.php?number=3219
    Angiosarcoma is a highly aggressive mesenchymal tumor. Although systemic chemotherapy is often considered for the inoperable or metastatic angiosarcoma, the outcome of such treatment is unsatisfactory and poorly delineated. […] The prognosis of angiosarcoma is generally poor even in localized disease with 5-year overall survival (OS) up to only 60% with a median survival of around 310 months for metastatic disease. […] Patients with primary hepatic angiosarcoma tended to have poorer OS than those without liver involvement. […] Presence of liver involvement was significantly associated with poor OS. […] In the univariate Cox proportional hazard regression analysis, ECOG PS and presence of liver involvement were significantly associated with poor OS. […] In addition, higher pre-chemotherapy bilirubin and lower pre-chemotherapy albumin were associated with poor OS. […] Treatment with first-line wPac and later-line pazopanib seems to provide survival benefit, especially for patients with advanced angiosarcoma without liver involvement.
  • #66 Primary splenic angiosarcoma: a case series of a rare oncological entity and diagnostic challenge
    https://medicaljournalssweden.se/actaoncologica/article/download/35412/45905?inline=1
    For PAS specifically, there are no guidelines or trial data available. The second patient presented here was treated with paclitaxel weekly for a metastatic PAS. […] In conclusion, primary splenic angiosarcoma is a rare and aggressive neoplasm with challenging diagnostic and management aspects. Awareness for the disease, its nonspecific clinical presentation, and large overlap with benign lesions on various imaging modalities is essential to make a timely diagnosis. Incorporating [18F]FDG-PET/CT into the diagnostic algorithm may aid in differentiating between benign and malignant lesions. Whenever possible early splenectomy is recommended. Paclitaxel may be considered as a first-line systemic treatment option. Emerging immunotherapy and tyrosine kinase inhibitor treatment show potential, but more data are needed to evaluate their effectiveness. For now, PAS remains a very aggressive disease for which currently available systemic therapies have only marginal activity.
  • #67 Survival predictors of metastatic angiosarcomas: a surveillance, epidemiology, and end results program population-based retrospective study | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-020-07300-7
    Angiosarcomas (AS) have poor prognosis and often metastasize to distant sites. The potential predictors of metastatic angiosarcomas (MAS) have not been extensively investigated. The main objective of this study was to identify survival predictors of MAS. […] Overall, 96 patients (33.8%) had two or more metastatic sites. The 1- and 3-year overall survival (OS) rates were 20.8 and 3.8% while 1- and 3-year cancer-specific survival (CSS) rates were 22.0 and 5.2%, respectively. […] MAS is associated with extremely poor survival. Chemotherapy, RT, and tumor size are independent predictors of OS. Chemotherapy and tumor size are independent prognostic factors of CSS. […] In this study, we analyzed the survival predictors of MAS, known for its extremely poor survival rates, in 284 patients. Chemotherapy, RT and tumor size 10cm were identified as independent protective predictors of OS. Chemotherapy and tumor size 10cm were independent favorable prognostic factors of CSS. Grade IV was associated with poorer survival of CSS. The number of metastatic sites did not appear to affect OS and CSS. Based on the collective findings, we recommend chemotherapy as the preferred treatment option for MAS patients.
  • #68 Angiosarcoma: clinical outcomes and prognostic factors, a single-center analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC11196347/
    The median OS of 19.3 months and 1 year OS of 57.5% reported herein are comparable to previous cohort studies on localized and metastatic AS. […] In our study, combination therapies (surgery with RT or surgery with CTX vs. surgery alone) were not significantly associated with OS or LPFS. […] In our cohort, locally recurrent disease at presentation was significantly associated with LPFS and not significantly associated with OS. […] Tumor size of 5 cm is repeatedly described as another adverse prognostic factor for OS and disease-specific survival in AS patients. […] Older age and metastatic disease at initial presentation status were adverse prognostic factors for local recurrence and OS in our study cohort.
  • #69 Prognostic and predictive factors for angiosarcoma patients receiving paclitaxel once weekly plus or minus bevacizumab: an ancillary study derived from a randomized clinical trial | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4828-1
    Among the 51 patients enrolled and treated in this trial, biomarker analysis was performed for 42: 18 in Arm A (single-agent) and 24 in Arm B (combination). […] According to univariate analysis, factors associated with a poor PFS were as follows: visceral angiosarcoma, de novo angiosarcoma, and high PlGF and low VEGF-C baseline values. In multivariate analysis, de novo angiosarcoma (HR=2.5; p=0.024) and baseline VEGF-C value (HR=0.7; p=0.003) were significant prognostic factors. […] De novo angiosarcoma and a low baseline level of VEGF-C were found to be associated with a poor prognosis. […] The results of the present study confirmed that adding bevacizumab to weekly paclitaxel did not improve the PFS of advanced angiosarcoma. […] At baseline, high values of circulating PlGF and low values of VEGF-C were associated with poor outcomes.
  • #70 Prognostic nomograms for predicting overall survival and cancer-specific survival in patients with angiosarcoma, a SEER population-based study | Scientific Reports
    https://www.nature.com/articles/s41598-022-07444-5
    Angiosarcoma (AS) is a kind of highly aggressive cancer with high occurrence and mortality rates. This study aimed to establish a comprehensive and validated prognostic nomogram with various clinical indicators in non-metastatic AS patients after surgery. […] After the multivariate Cox regression analysis, gender, AJCC stage group 7th ed, T, N stage 7th ed, histologic grade and primary site were statistically identified as independent factors with OS and CSS (P<0.05). [...] The C-index of the nomograms for OS and CCS in the training cohort was 0.757 (95%CI 0.6970.817) and 0.762 (95%CI 0.7020.822), meanwhile, the C-index of those in the validation cohort was 0.749 (95%CI 0.6680.830) and 0.756 (95%CI 0.6760.836) respectively. [...] The results of calibration plots and ROC curve showed the nomograms qualified to measure the risk and prognosis.
  • #71 Prognostic nomograms for predicting overall survival and cancer-specific survival in patients with angiosarcoma, a SEER population-based study | Scientific Reports
    https://www.nature.com/articles/s41598-022-07444-5
    It has been reported that the median overall survival (OS) varied from 30 to 50 months for AS patients, resulting in a poor prognosis. […] Based on the univariate and multivariate regression, gender, age, AJCC stage group 7th ed, T and N stage 7th ed, histologic grade and primary site were recognized as the independent predictors of prognosis which were utilized to establish nomograms. […] The C-index of the model in the internal and external validation were all greater than 0.7 for OS (0.757, 0.749) and CSS (0.762, 0.756). […] This nomogram provides important clinical use in AS which are listed as follows. Firstly, Clinicians can more accurately and faster to estimate the survival chances and recognize the personalized risk of AS patients so as to adopt corresponding interventions. […] Taken together, our study constructed a novel predictive model to identify the risk in post-operative AS patients with no metastasis. The nomograms were capable to predict 3- and 5-year OS and CSS.
  • #72
    https://journals.lww.com/md-journal/fulltext/2025/01030/trends_in_the_incidence,_survival,_and_prognostic.53.aspx
    This study aimed to investigate the epidemiological trends of angiosarcoma and to establish a tool to estimate its prognosis. […] The median survival time is approximately 30 to 50 months, with a 5-year survival rate of 10% to 50%. […] In multivariable analyses, age, sex, marital status, grade, historical stage, surgery, site, and tumor size were independent prognostic factors for angiosarcoma. […] The concordance index of the nomogram was significantly higher than that of the American Joint Committee on Cancer (AJCC) 6th edition and the AJCC 7th edition (0.74 vs 0.61 vs 0.66, respectively). […] The median OS was 16 months and the 1-, 3-, and 5-year survival rates were 55.2%, 33.7%, and 26.4%, respectively. […] In general, patients with local and stage G1 disease have better survival rates. Overall, patients benefited from surgery (HR, 1.73; 95% CI: 1.5971.874; P.05). […] The nomogram showed a good prediction accuracy. […] The C-index of the nomogram for OS was 0.74 (95% CI: 0.730.75). […] The C-index of our nomogram was significantly better than those of the 6th and 7th editions of the AJCC staging system, indicating the superiority of our nomogram.
  • #73 Prognostic nomograms for predicting overall survival and cancer-specific survival in patients with angiosarcoma, a SEER population-based study | Scientific Reports
    https://www.nature.com/articles/s41598-022-07444-5
    Angiosarcoma (AS) is a kind of highly aggressive cancer with high occurrence and mortality rates. This study aimed to establish a comprehensive and validated prognostic nomogram with various clinical indicators in non-metastatic AS patients after surgery. […] After the multivariate Cox regression analysis, gender, AJCC stage group 7th ed, T, N stage 7th ed, histologic grade and primary site were statistically identified as independent factors with OS and CSS (P<0.05). [...] The C-index of the nomograms for OS and CCS in the training cohort was 0.757 (95%CI 0.6970.817) and 0.762 (95%CI 0.7020.822), meanwhile, the C-index of those in the validation cohort was 0.749 (95%CI 0.6680.830) and 0.756 (95%CI 0.6760.836) respectively. [...] The results of calibration plots and ROC curve showed the nomograms qualified to measure the risk and prognosis.
  • #74
    https://journals.lww.com/md-journal/fulltext/2025/01030/trends_in_the_incidence,_survival,_and_prognostic.53.aspx
    This study aimed to investigate the epidemiological trends of angiosarcoma and to establish a tool to estimate its prognosis. […] The median survival time is approximately 30 to 50 months, with a 5-year survival rate of 10% to 50%. […] In multivariable analyses, age, sex, marital status, grade, historical stage, surgery, site, and tumor size were independent prognostic factors for angiosarcoma. […] The concordance index of the nomogram was significantly higher than that of the American Joint Committee on Cancer (AJCC) 6th edition and the AJCC 7th edition (0.74 vs 0.61 vs 0.66, respectively). […] The median OS was 16 months and the 1-, 3-, and 5-year survival rates were 55.2%, 33.7%, and 26.4%, respectively. […] In general, patients with local and stage G1 disease have better survival rates. Overall, patients benefited from surgery (HR, 1.73; 95% CI: 1.5971.874; P.05). […] The nomogram showed a good prediction accuracy. […] The C-index of the nomogram for OS was 0.74 (95% CI: 0.730.75). […] The C-index of our nomogram was significantly better than those of the 6th and 7th editions of the AJCC staging system, indicating the superiority of our nomogram.
  • #75 Prognostic nomograms for predicting overall survival and cancer-specific survival in patients with angiosarcoma, a SEER population-based study | Scientific Reports
    https://www.nature.com/articles/s41598-022-07444-5
    It has been reported that the median overall survival (OS) varied from 30 to 50 months for AS patients, resulting in a poor prognosis. […] Based on the univariate and multivariate regression, gender, age, AJCC stage group 7th ed, T and N stage 7th ed, histologic grade and primary site were recognized as the independent predictors of prognosis which were utilized to establish nomograms. […] The C-index of the model in the internal and external validation were all greater than 0.7 for OS (0.757, 0.749) and CSS (0.762, 0.756). […] This nomogram provides important clinical use in AS which are listed as follows. Firstly, Clinicians can more accurately and faster to estimate the survival chances and recognize the personalized risk of AS patients so as to adopt corresponding interventions. […] Taken together, our study constructed a novel predictive model to identify the risk in post-operative AS patients with no metastasis. The nomograms were capable to predict 3- and 5-year OS and CSS.
  • #76
    https://journals.lww.com/md-journal/fulltext/2025/01030/trends_in_the_incidence,_survival,_and_prognostic.53.aspx
    This study aimed to investigate the epidemiological trends of angiosarcoma and to establish a tool to estimate its prognosis. […] The median survival time is approximately 30 to 50 months, with a 5-year survival rate of 10% to 50%. […] In multivariable analyses, age, sex, marital status, grade, historical stage, surgery, site, and tumor size were independent prognostic factors for angiosarcoma. […] The concordance index of the nomogram was significantly higher than that of the American Joint Committee on Cancer (AJCC) 6th edition and the AJCC 7th edition (0.74 vs 0.61 vs 0.66, respectively). […] The median OS was 16 months and the 1-, 3-, and 5-year survival rates were 55.2%, 33.7%, and 26.4%, respectively. […] In general, patients with local and stage G1 disease have better survival rates. Overall, patients benefited from surgery (HR, 1.73; 95% CI: 1.5971.874; P.05). […] The nomogram showed a good prediction accuracy. […] The C-index of the nomogram for OS was 0.74 (95% CI: 0.730.75). […] The C-index of our nomogram was significantly better than those of the 6th and 7th editions of the AJCC staging system, indicating the superiority of our nomogram.
  • #77 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #78 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    In summary, angiosarcomas are a family of sarcomas with distinct clinical behavior depending on the primary sites, which effects the clinical outcome. Tumor recurrence/metastasis, tumor differentiation and treatment modality are the independent factors for prognosis. In the present study, multimodal treatment may improve the clinical outcome of patients with angiosarcoma.
  • #79 Prognostic nomograms for predicting overall survival and cancer-specific survival in patients with angiosarcoma, a SEER population-based study | Scientific Reports
    https://www.nature.com/articles/s41598-022-07444-5
    It has been reported that the median overall survival (OS) varied from 30 to 50 months for AS patients, resulting in a poor prognosis. […] Based on the univariate and multivariate regression, gender, age, AJCC stage group 7th ed, T and N stage 7th ed, histologic grade and primary site were recognized as the independent predictors of prognosis which were utilized to establish nomograms. […] The C-index of the model in the internal and external validation were all greater than 0.7 for OS (0.757, 0.749) and CSS (0.762, 0.756). […] This nomogram provides important clinical use in AS which are listed as follows. Firstly, Clinicians can more accurately and faster to estimate the survival chances and recognize the personalized risk of AS patients so as to adopt corresponding interventions. […] Taken together, our study constructed a novel predictive model to identify the risk in post-operative AS patients with no metastasis. The nomograms were capable to predict 3- and 5-year OS and CSS.
  • #80 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/276512-overview
    All angiosarcomas tend to be aggressive and are often multicentric. These tumors have high rates of local recurrence and metastasis because of their intrinsic biologic properties and because they are often misdiagnosed, leading to a poor prognosis and a high mortality rate. Malignant vascular tumors are clinically aggressive, difficult to treat, and have a reported 5-year survival rate around 20-35%. […] Advanced stage at presentation and lack of radical or wide excision are associated with higher recurrence rates, higher distant metastasis rates, and worsened survival. This is in contrast to the 5-year survival for all types of soft tissue sarcomas, which is around 65%. […] Angiosarcoma of the viscera (particularly liver and heart) and retroperitoneal disease are especially associated with poor outcome.
  • #81 Primary splenic angiosarcoma: a case series of a rare oncological entity and diagnostic challenge
    https://medicaljournalssweden.se/actaoncologica/article/download/35412/45905?inline=1
    Primary angiosarcoma of the spleen (PAS), an exceptionally rare and aggressive neoplasm with high metastatic risk (70%-85%), is frequently diagnosed in an advanced or metastatic stage. […] Unfortunately, the prognosis is dismal with a median overall survival (OS) of only 5-6 months. In case of early detection, splenectomy has been reported to increase the median OS to 14 months. […] While it is crucial to acknowledge that further trial data is required to evaluate the efficacy of emerging treatment regimens, designing and conducting trials for PAS is challenging given its scarcity and aggressive behavior. […] When diagnosed early, the recommended primary treatment for PAS is splenectomy before any potential splenic rupture occurs, as it is the only treatment that benefits OS. It should be noted however, that splenectomy is rarely curative.
  • #82 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #83 Survival predictors of metastatic angiosarcomas: a surveillance, epidemiology, and end results program population-based retrospective study | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-020-07300-7
    Angiosarcomas (AS) have poor prognosis and often metastasize to distant sites. The potential predictors of metastatic angiosarcomas (MAS) have not been extensively investigated. The main objective of this study was to identify survival predictors of MAS. […] Overall, 96 patients (33.8%) had two or more metastatic sites. The 1- and 3-year overall survival (OS) rates were 20.8 and 3.8% while 1- and 3-year cancer-specific survival (CSS) rates were 22.0 and 5.2%, respectively. […] MAS is associated with extremely poor survival. Chemotherapy, RT, and tumor size are independent predictors of OS. Chemotherapy and tumor size are independent prognostic factors of CSS. […] In this study, we analyzed the survival predictors of MAS, known for its extremely poor survival rates, in 284 patients. Chemotherapy, RT and tumor size 10cm were identified as independent protective predictors of OS. Chemotherapy and tumor size 10cm were independent favorable prognostic factors of CSS. Grade IV was associated with poorer survival of CSS. The number of metastatic sites did not appear to affect OS and CSS. Based on the collective findings, we recommend chemotherapy as the preferred treatment option for MAS patients.
  • #84 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    In summary, angiosarcomas are a family of sarcomas with distinct clinical behavior depending on the primary sites, which effects the clinical outcome. Tumor recurrence/metastasis, tumor differentiation and treatment modality are the independent factors for prognosis. In the present study, multimodal treatment may improve the clinical outcome of patients with angiosarcoma.
  • #85 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #86 Primary splenic angiosarcoma: a case series of a rare oncological entity and diagnostic challenge
    https://medicaljournalssweden.se/actaoncologica/article/download/35412/45905?inline=1
    Primary angiosarcoma of the spleen (PAS), an exceptionally rare and aggressive neoplasm with high metastatic risk (70%-85%), is frequently diagnosed in an advanced or metastatic stage. […] Unfortunately, the prognosis is dismal with a median overall survival (OS) of only 5-6 months. In case of early detection, splenectomy has been reported to increase the median OS to 14 months. […] While it is crucial to acknowledge that further trial data is required to evaluate the efficacy of emerging treatment regimens, designing and conducting trials for PAS is challenging given its scarcity and aggressive behavior. […] When diagnosed early, the recommended primary treatment for PAS is splenectomy before any potential splenic rupture occurs, as it is the only treatment that benefits OS. It should be noted however, that splenectomy is rarely curative.
  • #87 Angiosarcoma – Rare Cancers Australia
    http://knowledge.rarecancers.org.au/knowledgebase/cancer-types/237/angiosarcoma
    Angiosarcomas are rare types of sarcomas (cancers arising from bone or soft tissue) that develop in the lining of blood vessels (haemangiosarcoma) or lymph vessels (lymphangiosarcomas) throughout the body. […] It is often aggressive, and may not carry as good of a prognosis as other types of cancer. […] These cancers can often be misdiagnosed, are often aggressive, and may not have as good of a prognosis as other types of angiosarcoma. […] Breast angiosarcomas are often more aggressive than other types of breast cancers, and may not have as good of a prognosis as other breast cancers. […] Angiosarcomas of deep soft tissues are often aggressive, and can have varied prognoses based on tumour location. […] Radiation-induced angiosarcoma can be aggressive, and may not have as good of a prognosis as other types of angiosarcoma.
  • #88 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    The results revealed in the present study suggest that angiosarcoma is a family of sarcomas with distinctive behavior depending on the primary site. According to the primary site, all the cases in the present study were divided into four groups: i) Head and neck; ii) breast; iii) viscera (including internal organ and bone); and iv) soft tissue (including trunk and extremities). Overall, angiosarcomas exhibited a similar distribution between sexes (female/male, 103/97; ratio, 1.1:1). […] The results of the present study also demonstrated that the primary sites of the tumor affect the prognosis, which may be used to classify angiosarcoma. DFS and OS were affected by the primary site of angiosarcoma in the present study. The head and neck angiosarcomas were associated with the poorest survival, with a 5-year OS of 28.0%. However, the outcomes of patients with breast angiosarcomas in the present study were favorable, with a 5-year OS of 87.5%.
  • #89 Prognostic and predictive factors for angiosarcoma patients receiving paclitaxel once weekly plus or minus bevacizumab: an ancillary study derived from a randomized clinical trial | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4828-1
    Among the 51 patients enrolled and treated in this trial, biomarker analysis was performed for 42: 18 in Arm A (single-agent) and 24 in Arm B (combination). […] According to univariate analysis, factors associated with a poor PFS were as follows: visceral angiosarcoma, de novo angiosarcoma, and high PlGF and low VEGF-C baseline values. In multivariate analysis, de novo angiosarcoma (HR=2.5; p=0.024) and baseline VEGF-C value (HR=0.7; p=0.003) were significant prognostic factors. […] De novo angiosarcoma and a low baseline level of VEGF-C were found to be associated with a poor prognosis. […] The results of the present study confirmed that adding bevacizumab to weekly paclitaxel did not improve the PFS of advanced angiosarcoma. […] At baseline, high values of circulating PlGF and low values of VEGF-C were associated with poor outcomes.
  • #90 Prognostic and predictive factors for angiosarcoma patients receiving paclitaxel once weekly plus or minus bevacizumab: an ancillary study derived from a randomized clinical trial | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4828-1
    Finally, an increase in FGF between day 1 and day 8 was associated with a heightened risk of disease progression. […] When treated with weekly paclitaxel, the outcome of angiosarcoma patients in advanced stages is better for radiation-induced tumors than it is for de novo angiosarcoma. […] A low level of circulating VEGF-C and a high level of circulating PlGF at baseline were found to be associated with a poor outcome.
  • #91 Prognostic and predictive factors for angiosarcoma patients receiving paclitaxel once weekly plus or minus bevacizumab: an ancillary study derived from a randomized clinical trial | BMC Cancer | Full Text
    https://bmccancer.biomedcentral.com/articles/10.1186/s12885-018-4828-1
    Finally, an increase in FGF between day 1 and day 8 was associated with a heightened risk of disease progression. […] When treated with weekly paclitaxel, the outcome of angiosarcoma patients in advanced stages is better for radiation-induced tumors than it is for de novo angiosarcoma. […] A low level of circulating VEGF-C and a high level of circulating PlGF at baseline were found to be associated with a poor outcome.
  • #92 Angiosarcoma – Rare Cancers Australia
    http://knowledge.rarecancers.org.au/knowledgebase/cancer-types/237/angiosarcoma
    Angiosarcomas are rare types of sarcomas (cancers arising from bone or soft tissue) that develop in the lining of blood vessels (haemangiosarcoma) or lymph vessels (lymphangiosarcomas) throughout the body. […] It is often aggressive, and may not carry as good of a prognosis as other types of cancer. […] These cancers can often be misdiagnosed, are often aggressive, and may not have as good of a prognosis as other types of angiosarcoma. […] Breast angiosarcomas are often more aggressive than other types of breast cancers, and may not have as good of a prognosis as other breast cancers. […] Angiosarcomas of deep soft tissues are often aggressive, and can have varied prognoses based on tumour location. […] Radiation-induced angiosarcoma can be aggressive, and may not have as good of a prognosis as other types of angiosarcoma.
  • #93 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    The results revealed in the present study suggest that angiosarcoma is a family of sarcomas with distinctive behavior depending on the primary site. According to the primary site, all the cases in the present study were divided into four groups: i) Head and neck; ii) breast; iii) viscera (including internal organ and bone); and iv) soft tissue (including trunk and extremities). Overall, angiosarcomas exhibited a similar distribution between sexes (female/male, 103/97; ratio, 1.1:1). […] The results of the present study also demonstrated that the primary sites of the tumor affect the prognosis, which may be used to classify angiosarcoma. DFS and OS were affected by the primary site of angiosarcoma in the present study. The head and neck angiosarcomas were associated with the poorest survival, with a 5-year OS of 28.0%. However, the outcomes of patients with breast angiosarcomas in the present study were favorable, with a 5-year OS of 87.5%.
  • #94 Azthena logo with the word Azthena
    https://www.news-medical.net/health/Angiosarcoma-Prognosis.aspx
    Angiosarcoma patients are mostly diagnosed after the tumor has spread all over the body and this type of late identification may result in a poor prognosis. According to various studies, improved prognosis is achieved when patients have smaller tumors that can be easily removed from the body. […] Better prognosis is usually seen in low-grade breast angiosarcoma. […] Up to 50% of patients show distant spread of the disease in the body at the time of diagnosis. […] Smaller tumors in sarcoma patients are easily removed by radiotherapy, chemotherapy, and surgery. […] The worst prognosis is seen mainly in soft tissue angiosarcomas, because they are very difficult to examine. […] Angiosarcoma of breast has a comparatively better prognosis. […] Generally, angiosarcomas have a poor prognosis.
  • #95 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/276512-overview
    In a study of 346 primary hepatic angiosarcoma (PHA) patients, the median survival was 1.9 months. Those who underwent surgical resection had a higher median survival rate of 7.7 months. Chemotherapy treatment also increased median survival to 5.1 months. […] More than 50% of patients develop metastatic disease, with the lung the primary organ involved. […] Angiosarcoma of the soft tissue is a high-grade sarcoma with a high rate of death and short survival time. A large number of patients, 50% in some series, also had metastasis, and a significant number (20%) had local recurrences. […] Older patient age, retroperitoneum location, and larger tumor size are unfavorable prognostic factors. Approximately 66% of retroperitoneal angiosarcomas recur locally in the tumor bed and can recur diffusely throughout the peritoneal cavity (angiosarcomatosis).
  • #96 Real-World Clinical Outcomes and Prognostic Factors for Patients with Advanced Angiosarcoma who Received Systemic Treatment
    https://e-crt.org/journal/view.php?number=3219
    Angiosarcoma is a highly aggressive mesenchymal tumor. Although systemic chemotherapy is often considered for the inoperable or metastatic angiosarcoma, the outcome of such treatment is unsatisfactory and poorly delineated. […] The prognosis of angiosarcoma is generally poor even in localized disease with 5-year overall survival (OS) up to only 60% with a median survival of around 310 months for metastatic disease. […] Patients with primary hepatic angiosarcoma tended to have poorer OS than those without liver involvement. […] Presence of liver involvement was significantly associated with poor OS. […] In the univariate Cox proportional hazard regression analysis, ECOG PS and presence of liver involvement were significantly associated with poor OS. […] In addition, higher pre-chemotherapy bilirubin and lower pre-chemotherapy albumin were associated with poor OS. […] Treatment with first-line wPac and later-line pazopanib seems to provide survival benefit, especially for patients with advanced angiosarcoma without liver involvement.
  • #97 Primary splenic angiosarcoma: a case series of a rare oncological entity and diagnostic challenge
    https://medicaljournalssweden.se/actaoncologica/article/download/35412/45905?inline=1
    Primary angiosarcoma of the spleen (PAS), an exceptionally rare and aggressive neoplasm with high metastatic risk (70%-85%), is frequently diagnosed in an advanced or metastatic stage. […] Unfortunately, the prognosis is dismal with a median overall survival (OS) of only 5-6 months. In case of early detection, splenectomy has been reported to increase the median OS to 14 months. […] While it is crucial to acknowledge that further trial data is required to evaluate the efficacy of emerging treatment regimens, designing and conducting trials for PAS is challenging given its scarcity and aggressive behavior. […] When diagnosed early, the recommended primary treatment for PAS is splenectomy before any potential splenic rupture occurs, as it is the only treatment that benefits OS. It should be noted however, that splenectomy is rarely curative.
  • #98 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #99 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    In summary, angiosarcomas are a family of sarcomas with distinct clinical behavior depending on the primary sites, which effects the clinical outcome. Tumor recurrence/metastasis, tumor differentiation and treatment modality are the independent factors for prognosis. In the present study, multimodal treatment may improve the clinical outcome of patients with angiosarcoma.
  • #100 Clinicopathological features and prognostic factors in angiosarcoma: A retrospective analysis of 200 patients from a single Chinese medical institute
    https://www.spandidos-publications.com/10.3892/ol.2017.6892
    Tumor size and tumor differentiation are important prognostic factors in soft tissue sarcoma, and their value in angiosarcoma may also be confirmed in the present study. The present univariate analysis confirmed that tumor size and histological grade are prognostic factors for DFS and OS. Tumors size 5 cm and high-grade histological differentiation predicted poor prognosis, as previously described. […] The present study identified that tumor differentiation and recurrence/metastasis were independent prognostic factors using multivariate survival analysis. Although certain parameters may not be independent prognostic factors, the present study hypothesizes that all the aforementioned factors mentioned should be considered by physicians when assessing the biological behavior of the tumor and choosing the therapeutic strategies.
  • #101 Angiosarcoma: clinical outcomes and prognostic factors, a single-center analysis
    https://pmc.ncbi.nlm.nih.gov/articles/PMC11196347/
    This study sought to investigate oncological outcomes and prognostic factors for patients with angiosarcomas (AS). […] Overall survival (OS), local control (LC) and local progression-free survival (LPFS) were assessed by Kaplan-Meier estimator. […] Multivariable Cox regression of OS showed a significant association with age per year (hazard ratio (HR): 1.03, p=0.044) and metastatic disease at presentation (hazard ratio: 3.24, p=0.015). For LPFS, age per year (HR: 1.04, p=0.008), locally recurrent disease at presentation (HR: 5.32, p=0.013), and metastatic disease at presentation (HR: 4.06, p=0.009) had significant associations. […] Older age and metastatic disease at initial presentation status were negatively associated with OS and LPFS. […] Despite intensive treatment, the overall prognosis of patients is limited. Age and metastatic disease at presentation were significant adverse prognostic factors for OS, and local disease progression after therapy.
  • #102 Angiosarcoma Outcomes and Prognostic Factors: A 25-Year Single Institution Experience
    https://pmc.ncbi.nlm.nih.gov/articles/PMC3664266/
    Angiosarcoma is an aggressive malignancy with endothelial differentiation and notoriously poor prognosis despite aggressive therapy. […] The 5-year overall survival (OS) was 40% with a median of 16 months. […] Our large single institution series confirms the poor prognosis of angiosarcoma, supports a central role for surgical excision in management and highlights the need for novel therapies particularly in patients who present with metastatic disease. […] The prognostic significance of tumor factors is shown in Figure 3. Metastatic disease at presentation was a critical adverse prognostic factor (median OS of 3 months vs. 60 months in patients with localized disease). […] The site of the primary lesion significantly influenced survival (median OS of 5 months in patients with visceral/deep soft tissue angiosarcoma vs. 60 months in patients with non-visceral/deep primaries).
  • #103 Primary splenic angiosarcoma: a case series of a rare oncological entity and diagnostic challenge
    https://medicaljournalssweden.se/actaoncologica/article/download/35412/45905?inline=1
    For PAS specifically, there are no guidelines or trial data available. The second patient presented here was treated with paclitaxel weekly for a metastatic PAS. […] In conclusion, primary splenic angiosarcoma is a rare and aggressive neoplasm with challenging diagnostic and management aspects. Awareness for the disease, its nonspecific clinical presentation, and large overlap with benign lesions on various imaging modalities is essential to make a timely diagnosis. Incorporating [18F]FDG-PET/CT into the diagnostic algorithm may aid in differentiating between benign and malignant lesions. Whenever possible early splenectomy is recommended. Paclitaxel may be considered as a first-line systemic treatment option. Emerging immunotherapy and tyrosine kinase inhibitor treatment show potential, but more data are needed to evaluate their effectiveness. For now, PAS remains a very aggressive disease for which currently available systemic therapies have only marginal activity.
  • #104 Prognostic nomograms for predicting overall survival and cancer-specific survival in patients with angiosarcoma, a SEER population-based study | Scientific Reports
    https://www.nature.com/articles/s41598-022-07444-5
    It has been reported that the median overall survival (OS) varied from 30 to 50 months for AS patients, resulting in a poor prognosis. […] Based on the univariate and multivariate regression, gender, age, AJCC stage group 7th ed, T and N stage 7th ed, histologic grade and primary site were recognized as the independent predictors of prognosis which were utilized to establish nomograms. […] The C-index of the model in the internal and external validation were all greater than 0.7 for OS (0.757, 0.749) and CSS (0.762, 0.756). […] This nomogram provides important clinical use in AS which are listed as follows. Firstly, Clinicians can more accurately and faster to estimate the survival chances and recognize the personalized risk of AS patients so as to adopt corresponding interventions. […] Taken together, our study constructed a novel predictive model to identify the risk in post-operative AS patients with no metastasis. The nomograms were capable to predict 3- and 5-year OS and CSS.
  • #105
    https://journals.lww.com/md-journal/fulltext/2025/01030/trends_in_the_incidence,_survival,_and_prognostic.53.aspx
    This study aimed to investigate the epidemiological trends of angiosarcoma and to establish a tool to estimate its prognosis. […] The median survival time is approximately 30 to 50 months, with a 5-year survival rate of 10% to 50%. […] In multivariable analyses, age, sex, marital status, grade, historical stage, surgery, site, and tumor size were independent prognostic factors for angiosarcoma. […] The concordance index of the nomogram was significantly higher than that of the American Joint Committee on Cancer (AJCC) 6th edition and the AJCC 7th edition (0.74 vs 0.61 vs 0.66, respectively). […] The median OS was 16 months and the 1-, 3-, and 5-year survival rates were 55.2%, 33.7%, and 26.4%, respectively. […] In general, patients with local and stage G1 disease have better survival rates. Overall, patients benefited from surgery (HR, 1.73; 95% CI: 1.5971.874; P.05). […] The nomogram showed a good prediction accuracy. […] The C-index of the nomogram for OS was 0.74 (95% CI: 0.730.75). […] The C-index of our nomogram was significantly better than those of the 6th and 7th editions of the AJCC staging system, indicating the superiority of our nomogram.