Materiały źródłowe

• #1 Angiosarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK441983/

  Angiosarcoma (AS) comprises 1% of all soft-tissue sarcoma (STS), which are themselves a rare malignancy. […] AS comprises up to 2% of all STS and 5-4% of all cutaneous STS. […] AS has a similar distribution between sexes; however, is more common in elderly white men. […] It can develop in any body organ, but cutaneous AS occurs more commonly in the head and neck region, particularly in the scalp.

• #2 Angiosarcoma: a review of diagnosis and current treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6895451/

  Angiosarcoma is a highly malignancy of endothelial tumor and accounts about 2-3% of all adult soft tissue sarcomas. This tumor can occur in any location of the body, but has a notably predilection for the skin and superficial soft tissue. The cutaneous angiosarcoma makes up two-thirds of all cases. Due to its aggressive nature with the tendency to distant organ metastasize, the lung and brain are the most common sites of metastasize with a poor prognosis. Previous studies reported that the 5-year survival rate of angiosarcoma patients was among 30-40% and the OS ranging from 6 to 16 months. Up to 20-40% of patients suffered from the local recurrence or metastasis. According to the research, the distribution of angiosarcoma was tended to the older male individuals among 60-70 years old, especially in cutaneous angiosarcomas.

• #3 Angiosarcoma – NCI

  https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-vascular-tumors/angiosarcoma

  Angiosarcoma is a rare cancer that develops in the inner lining of blood vessels and lymph vessels. This cancer can occur anywhere in the body but most often is in the skin, breast, liver and spleen. […] For every million people, one will be diagnosed with angiosarcoma per year in the U.S. Angiosarcomas make up about 1% to 2% of all sarcomas. They are most common in people over the age of 70 but can happen at any age. […] Doctors estimate survival rates by how groups of people with angiosarcomas have done in the past. Given that there are so few angiosarcoma patients, survival rates may not be accurate. They also do not consider newer treatments being developed.

• #4 Incidence and site specific characteristics of angiosarcoma in Japan using a population-based national cancer registry from 2016 to 2019 | Scientific Reports

  https://www.nature.com/articles/s41598-025-94956-5

  Angiosarcoma is so rare, no reports on its epidemiology and prognosis using nationwide population-based data exist. The present study aimed to clarify the characteristics and clinical outcomes of angiosarcoma using the National Cancer Registry (NCR) in Japan. Angiosarcoma accounts for 8.4% of all STS (adjusted incidence: 0.18/100,000/year). The present study is the first to have clarified the epidemiology, clinical features, treatment, prognosis, and significant factors affecting prognosis of patients with angiosarcoma in Japan. […] The present study first identified the age-adjusted incidence of angiosarcoma in Japan as 0.18/100,000/year. The age-adjusted incidences of angiosarcoma were reported to be 0.26/100,000/year in France and 0.30/100,000/year in the United States, which are even higher compared with that in Japan.

• #5 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/276512-overview

  Angiosarcomas are rare neoplasms. Approximately 50% of angiosarcomas occur in the head and neck, but they account for less than 0.1% of head and neck malignancies. […] Around 2% of soft tissue sarcomas in general and 4% of cutaneous soft tissue sarcomas are angiosarcomas. […] A study of the Surveillance, Epidemiology, and End Results database found that the incidence of all soft tissue sarcomas from 1973 to 2006 was 5.9 per 100,000 persons. […] This would yield an incidence of approximately 1.2 per million for angiosarcomas. […] Worldwide incidence is also low; in the United Kingdom, for example, the National Cancer Intelligence Network (NCIN) reported that angiosarcomas represent 3.3% of all soft tissue sarcomas, with an incidence rate of 1.5 per million. […] Demographic variation includes the following: African Americans in the United States are rarely affected by cutaneous angiosarcoma.

• #6

  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 age-adjusted incidence of angiosarcoma gradually increased from 0.13/100,000 in 1975 to 0.33/100,000 in 2016 (annual percentage change [2.4]). […] The incidence and prevalence of angiosarcoma has increased over the past 40 years. […] The findings of this study will benefit clinical practice and future research in this area. […] The age-adjusted annual incidence has increased from 0.13/100,000 in 1975 to 0.33/100,000 in 2016, representing a 2.54-fold increase. […] We found that the increased prevalence rates were mainly attributable to angiosarcoma of the soft tissues, including the heart, skin, and breast. […] The overall 1-, 3-, and 5-year survival rates were 55.2%, 33.7%, and 26.4%, respectively, which is consistent with the results of previous studies.

• #7 Incidence and Presenting Characteristics of Angiosarcoma in the US, 2001-2020

  https://www.cancer.fr/professionnels-de-sante/veille/nota-bene-cancer/bulletin-n-602/incidence-and-presenting-characteristics-of-angiosarcoma-in-the-us-2001-2020

  Angiosarcoma is an aggressive vascular malignant neoplasm presenting either as a primary or secondary cancer, often arising after radiotherapy or in the context of preexisting lymphedema. […] Comprehensive data describing its incidence and presentation patterns are needed. […] The US incidence of angiosarcoma doubled between 2001 (657 cases) and 2019 (1312 cases), reflecting both an increase in the adjusted incidence rate of 1.6% per year (P=.001), to 3.3 cases per 1000000 person-years (95% CI, 3.1-3.5 cases per 1000000 person-years), and an increase in the population at risk. […] Overall, 72.3% of cases of angiosarcoma (n=13955) were cutaneous, subcutaneous, or breast angiosarcomas; 24.4% were visceral (n=4701); and 3.3% were located in unknown or rare primary sites (n=633). […] The incidence rate of visceral angiosarcoma was also found to be increasing (1.5% per year; P=.001). […] This cross-sectional study describes angiosarcoma presentation patterns and incidence rates in the US over a 20-year period and shows that the number of cases in men and women increased, with the greatest increase among women with secondary angiosarcoma of the chest, breast, and upper extremity.

• #8 Angiosarcoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/276512-overview

  Cutaneous angiosarcoma is more frequent in males than in females, with a male-to-female ratio of 2:1. […] Bone and soft tissue angiosarcoma have a similar sex distribution. […] Bone angiosarcoma appears most often in adults (second to seventh decades of life). […] Cutaneous angiosarcoma of the head and neck tends to occur in the elderly population.

• #9 Epidemiology, tumor characteristics and survival in patients with angiosarcoma in the United States: a population-based study of 4537 cases.

  https://www.epistemonikos.org/nl/documents/ad18b35b9957081c4bb6a6d67f9ce66774f3a61d

  OBJECTIVE: Limited by sample size, angiosarcoma was rarely studied. […] We aimed to investigate the characteristics and prognosis of angiosarcoma in the National Cancer Institute’s Surveillance, Epidemiology and End Results database. […] A total of 4537 patients with angiosarcoma were included with the median age of 69 years. […] The median overall survival was 82.1 (95% confidence interval: 76.5-87.7) months. […] Overall 1-, 2- and 5-year survival rates were 55.2 0.7, 41.0 0.7 and 26.3 0.7%, respectively. […] In the univariate analysis, age, gender, marital status, race, primary site, tumor grade, tumor size, Surveillance, Epidemiology and End Results historic stage and the surgery of primary site were significantly associated with overall survival. […] Multivariate Cox regression showed that factors including the patients older than 69 years, male, unmarried status, other primary sites, grades (III and IV), tumor size 5 cm, regional and distant stage and non-surgery were independently associated with poor survival.

• #10 Angiosarcoma

  https://atlasgeneticsoncology.org/solid-tumor/208914/angiosarcoma

  Angiosarcoma occurs across a wide age distribution and is most common in the elderly. Cutaneous angiosarcoma of the breast is frequently associated with prior radiation, and angiosarcoma can also arise in association with lymphedema or in pre-existing vascular malformations. […] The incidence of radiation-associated cutaneous angiosarcoma has increased with the widespread adoption of breast-conserving surgery with post-operative radiation to treat breast carcinoma. […] Angiosarcomas of deep soft tissue and viscera are relatively uncommon and occur in a wide age range, with a peak incidence in the 7th decade and with a male predominance.

• #11 Surgical Treatment of a Primary Angiosarcoma of the Breast | ACS

  https://www.facs.org/for-medical-professionals/news-publications/journals/case-reviews/issues/v4n2/14-plichta-primary-angiosarcoma-of-breast/

  Angiosarcoma is a malignant vascular neoplasm and the most common sarcoma of the breast. Primary angiosarcomas of the breast are rare tumors with a uniformly poor prognosis and ill-defined treatment guidelines. […] Compared to radiation-associated angiosarcoma, primary angiosarcoma of the breast has a younger onset and is typically diagnosed in women aged 30-40. […] Retrospective studies have shown that many women with primary angiosarcoma of the breast have lung metastases at the time of presentation, and as such, chest imaging is a critical component of the diagnostic workup. […] Among the treatment modalities evaluated, only wide surgical resection has been shown to significantly prolong survival in localized angiosarcoma. […] Despite surgery, angiosarcoma of the breast has a poor prognosis with an estimated five-year overall survival of 30% to 40%. […] Even when negative margins are achieved, there is a high risk of locally and distant recurrence. […] Given the high recurrence rates, close surveillance with imaging and chest wall exams is vital.

• #12

  https://link.springer.com/article/10.1007/s00428-024-03852-2

  Angiosarcoma (AS) of the breast represents a rare mesenchymal neoplasm, constituting a mere 0.2% of all malignant breast tumors. […] The global incidence of RAAS is on the rise. […] Importantly, the genetic profiles of these two forms differ significantly, with MYC amplification being a distinctive feature of RAAS rarely observed in the primary variant. […] In general, the prognosis for both forms remains poor. […] Multiple local recurrences are frequent in both forms, and the common metastatic sites include the lungs, liver, and bones. […] The median time interval between the diagnosis of breast carcinoma and the onset of RAAS was 8 years. […] The two manifestations of breast angiosarcoma exhibit distinctions in etiology, epidemiology, pathology, and genetic characteristics. […] Notably, primary AS predominantly affects younger women, with a median age of approximately 40 years.

• #13

  https://link.springer.com/article/10.1007/s00428-024-03852-2

  In contrast, secondary AS of the breast is a well-established rare and delayed complication arising from breast cancer treatment. […] Given that breast cancer typically afflicts the elderly, the peak incidence of RAAS occurs between 60 and 70 years, typically emerging 6 to 10 years after breast irradiation. […] In our studied cohort, we observed a similar age distribution, with a median age of 63 years. […] Secondary AS of the breast can both clinically and pathologically present as something else. […] RAAS usually exhibit distinctive morphological features (vessel-forming tumor) and occur in a specific clinical context (developing years after breast cancer treatment). […] However, it is crucial to emphasize that in cases where clinical data are unavailable or uncommunicated, and the vessel formation is not characteristic, a positive or doubtful TRPS1 immunohistochemical reaction may lead to an erroneous diagnostic conclusion. […] The genetic profiles of primary AS and RAAS exhibit notable distinctions. […] In summary, our study establishes that a notable proportion of breast AS expresses TRPS1, challenging the previously asserted high specificity of TRPS1 for breast carcinomas.

• #14 Liver angiosarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Liver_angiosarcoma

  Most people affected are 60-70 years old. Males are affected more often than women at a ratio of 3-4:1, although in children, girls are affected more commonly than boys. […] Although very rare with around 200 cases diagnosed each year, it is still considered the third most common primary liver cancer, making up around 2% of all primary liver cancers.

• #15 Hepatic angiosarcoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/hepatic-angiosarcoma?lang=us

  Hepatic angiosarcomas account for ~1% of primary liver tumors. They most commonly occur in patients 60-70 years of age with a male predominance (M:F = 4:1) 1-3. […] Prognosis is very poor, with survival uncommon beyond one year from diagnosis. […] Prognosis is poor with hepatic angiosarcomas being resistant to chemotherapy and radiation therapy. Median survival is less than 6 months and survival beyond 1 year is rare.

• #16 Primary Small Intestinal Angiosarcoma: Epidemiology, Diagnosis and Treatment | Nai | Journal of Clinical Medicine Research

  https://www.jocmr.org/index.php/JOCMR/article/view/3153

  Angiosarcoma is an aggressive mesenchymal sarcoma of endothelial cell origin with high mortality. Its occurrence in the small intestine is exceedingly low. […] Small bowel angiosarcoma occurs more often in men than women (1.6:1). The median age at diagnosis is 68.5 years. The overall median survival time is 150 days; the median survival time in female (300 days) is longer than that of male patients (120 days). […] The majority of the patients underwent surgical resection alone or surgery with subsequent chemotherapy. The patients treated with surgery plus chemotherapy survive longer than those underwent surgical resection only (median 420 days, n = 7 vs. 96.5 days, n = 26, respectively; P = 0.0275). Further studies of more cases are needed for a better understanding of this rare entity, as well as the development of effective strategies for prevention, early diagnosis, and treatment.

• #17 Breast Angiosarcoma Surveillance Study: UK national audit of management and outcomes of angiosarcoma of the breast and chest wall. — The Kennedy Institute of Rheumatology

  https://www.kennedy.ox.ac.uk/publications/1170083

  BACKGROUND: Breast angiosarcomas are rare tumours of vascular origin. Secondary angiosarcoma occurs following radiotherapy for breast cancer. Angiosarcomas have high recurrence and poor survival rates. This is concerning owing to the increasing use of adjuvant radiotherapy for the treatment of invasive breast cancer and ductal cancer in situ (DCIS), which could explain the rising incidence of angiosarcoma. […] Rates of recurrence were high with 14 of 28 (50 per cent) recurrences in patients with primary and 80 of 124 (64.5 per cent) in those with secondary angiosarcoma at 5 years. […] Development of secondary angiosarcoma had a negative impact on predicted breast cancer survival, with a median 10-year PREDICT prognostic rate of 69.6 per cent, compared with 54.0 per cent in the observed cohort. […] A detrimental impact of secondary angiosarcoma on breast cancer survival has been demonstrated.

• #18

  https://archbreastcancer.com/index.php/abc/article/view/758

  Post-radiation angiosarcoma (AS) of the breast, also known as radiation-associated angiosarcoma (RAAS) or radiation-induced angiosarcoma (RIAS), is a rare complication after breast radiotherapy. […] The annual incidence was 1 in 18,000 of the breast radiotherapy treated population. […] Post-radiation AS of the breast is a rare disease associated with a poor prognosis due to its aggressive nature and high recurrence rate. […] It is important to maintain a high index of suspicion in patients treated with past breast radiotherapy.

• #19 Evidence of Benign Vascular Anomalies Becoming Malignant Angiosarcoma Indicates Need for Increased Surveillance in Patients – ENTtoday

  https://www.enttoday.org/article/evidence-of-benign-vascular-anomalies-becoming-malignant-angiosarcoma-indicates-need-for-increased-surveillance-in-patients/

  Supportive evidence exists for the hypothesis that increased surveillance and an aggressive biopsy/surgical approach are warranted in patients with benign hemangioma whose size and characteristics change over time. […] These findings suggest the need for increased surveillance of patients with benign hemangioma whose size and characteristics change over time.

• #20 SEER Inquiry System – Question 20081002 Details

  https://seer.cancer.gov/seer-inquiry/inquiry-detail/20081002/

  Robbins Pathology states the following about liver angiosarcomas: Hepatic angiosarcomas are rare but of interest because they are associated with distinct carcinogens, including arsenic (exposure to arsenical pesticides), Thorocast (a radioactive contrast medium previously widely used in radiology), and polyvinyl chloride (PVC) (widely used in plastics). The increased frequency of angiosarcomas among works in the PVC industry is one of the truly well-documented instances of chemical carcinogenesis in humans. With all these agents, there is a very long latent period of many years between exposure and the development of tumors. […] Could the same apply to the spleen?

• #21

  https://www.cdc.gov/Mmwr/preview/mmwrhtml/00046136.htm

  Between September 1967 and December 1973, 4 cases of angiosarcoma of the liver were diagnosed among men employed in the polyvinyl chloride polymerization section of a B.F. Goodrich plant near Louisville, Kentucky. […] Angiosarcoma of the liver is an exceedingly rare tumor. It is estimated that only about 25 such cases occur each year in the United States. […] Epidemiologic studies have started to determine the extent of the problem in the United States, with respect both to angiosarcoma of the liver and to its possible relationship to post-toxic cirrhosis. […] This MMWR report on angiosarcoma of the liver in vinyl chloride polymerization workers represents a splendid description of a SHE(O). […] New cases of hepatic angiosarcoma in vinyl chloride polymerization workers have been virtually eliminated.

• #22

  https://www.orthobullets.com/pathology/8070/angiosarcoma

  Angiosarcomas are malignant, aggressive tumors that derive from the endothelium of blood vessels. […] The condition is typically seen in elderly patients who present with regional pain and overlying skin changes. […] Epidemiology […] Demographics […] elderly […] Anatomic location […] 60% long bone with osseous involvement. […] Risk factors […] chronic vascular stasis […] trauma […] exposure to polyvinyl chloride. […] Symptoms […] symptoms often insidious […] Physical exam […] hallmarked with […] overlying skin changes. […] Prognosis […] Poor prognosis […] high local failure rate and amputation is often required […] propensity for lymphatic spread […] metastases to lung is common.

• #23 Angiosarcoma | Springer Publishing

  https://connect.springerpub.com/content/book/978-0-8261-4853-7/part/part02/chapter/ch18

  Angiosarcoma (AS) is a rare tumor of endothelial origin. […] Furthermore, rate of local recurrence as well as metastasis is high, making AS a difficult cancer to treat. […] Regardless of the stage at presentation, as well as treatment, long-term prognosis is poor. […] Significantly more research must be performed in order to better understand the biology as well as the best means of treatment for AS.

• #24 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%.

• #25 Outcomes of head and neck angiosarcoma with different treatment modalities: a 20-year single institutional experience – Koh – Precision Cancer Medicine

  https://pcm.amegroups.org/article/view/6710/html

  Angiosarcoma (AS) is a rare cancer of the vascular endothelium, constituting 1.65.0% of all cutaneous soft tissue sarcomas. It commonly occurs in the head and neck region. The disease often metastasizes to regional nodes and distant sites (lung, liver and bone). As a result, the outcomes for head and neck AS are often poor, with 5-year survival ranging from 10% to 54%. […] Due to its rarity, there is a paucity of related literature. As most studies were presented as case reports and series, there is a lack of established management consensus. […] Our study demonstrated that AS is aggressive, as its 2-year (49.9%) and 5-year OS (18.9%) of localized disease were dismal. These findings were in line with some of the existing literature. However, our study had poorer outcomes as compared to Western reports.

• #26 Clinical characteristics of primary hepatic angiosarcoma outcomes: a SEER database analysis – Zeng – Translational Cancer Research

  https://tcr.amegroups.org/article/view/47875/html

  In total, 366 patients were included in this study. […] The prognosis of PHA was very poor, and the overall 6-month, 1-year and 2-year survival rates were 20.3%, 12.8% and 9.3%, respectively. […] PHA has a poor prognosis. Regular physical examinations are essential for the elderly. Patients aged 60 years could benefit from surgery. […] The prognosis of PHA was very poor, with a median survival of only 1 month. The overall 6-month, 1-year and 2-year survival rates were 20.3% (95% CI: 16.2-24.4%), 12.8% (95% CI: 9.3-16.3%) and 9.3% (95% CI: 6.2-12.4%), respectively. […] The delayed diagnosis and highly malignant characteristics of the tumor result in a very poor prognosis. […] The mainstay of treatment comprises radical tumor resection or hepatic resection when the patients’ general physical condition is adequate. […] In our study, we found that patients who received surgery had much better survival than those who did not, further supporting the choice of surgery as the primary treatment. […] We constructed accurate nomograms to predict survival that can greatly benefit clinicians.

• #27 Clinical characteristics associated with primary cardiac angiosarcoma outcomes: a surveillance, epidemiology and end result analysis | European Journal of Medical Research | Full Text

  https://eurjmedres.biomedcentral.com/articles/10.1186/s40001-019-0389-2

  Primary cardiac angiosarcoma (PCAS) is a rare type of tumour. Furthermore, descriptions of the demographic features and prognostic factors of PCAS patients have been poorly reported. A population cohort study was conducted using retrospectively extracted data from the SEER (Surveillance, Epidemiology and End Results) database for patients with histological diagnoses of PCAS; the extracted information included demographic, treatment and outcome data. A total of 168 cases of PCAS from 1973 to 2013 were included. The mean age at diagnosis was 44.415.5 years. PCAS was more prevalent in men than in women. The majority of PCAS patients were white (67.3%), while the incidence of PCAS in black individuals was relatively infrequent (19.0%). In addition, 87 cases were classified as distant stage, 44 as regional stage, and 33 as localized stage. The median disease-specific survival (DSS) was 7.22 months, and the 1-, 2- and 5-year DSS rate for PCAS patients was 34.7%, 14.3% and 10.2%, respectively. Further multivariate analyses showed that an age at (greater than or equal to) 45 years (HR2.165), no radiotherapy (HR1.629), tumour size 5 cm (HR3.182), and the summary stage was associated with worse PCAS-related survival. Cancer-directed surgery and radiotherapy significantly improved the DSS for patients with PCAS (P0.05). The C-index of the nomograms was 0.706 (95% CI 0.6540.758), and the calibration curves showed good agreement between the nomogram prediction and actual observation. PCAS is a rare cancer that is prone to have poor prognoses. To understand PCAS more thoroughly, more cases with adequate information are needed.

• #28 Clinical characteristics associated with primary cardiac angiosarcoma outcomes: a surveillance, epidemiology and end result analysis | European Journal of Medical Research | Full Text

  https://eurjmedres.biomedcentral.com/articles/10.1186/s40001-019-0389-2

  The incidence of PCAS during the 40-year study period ranged from 0.0069/100,000 (19761983) to 0.0163/100,000 (20082015). Additionally, the mortality rate shows a similar trend as the incidence rate. The average age of PCAS patients is 44.4 years, and the ratio of male to female patients is 1.3:1. This male-dominant tendency is in agreement with the data analysis results of other research studies in Eastern and Western countries. In our study, there are some factors related with PCAS survival. According to our data, the tumour grade is not associated with survival. […] The DSS rate at 1, 2, 3, 4, 5 and 10 years was 34.7%, 14.3%, 13.0%, 11.7%, 10.2% and 8.8%, respectively. The median DSS was 7.2 years. Furthermore, to predict the DSS of patients with PCAS, pre-treatment nomograms were established by a multivariate Cox regression model based on all meaningful variables that were factors for DSS. The C-index was estimated to be 0.706 (95% CI 0.6540.758).

• #29 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. […] Surveillance, Epidemiology, and End Results (SEER) datasets were used to identify patients with MAS from 2010 to 2016. […] 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. […] The majority of published studies to date, predominantly case series and individual institution analyses, have analyzed outcomes and prognosis for localized AS. […] In our study, multivariate analyses showed that age was not a significant independent predictor. […] 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.

• #30

  https://journals.lww.com/md-journal/fulltext/2025/01030/trends_in_the_incidence,_survival,_and_prognostic.53.aspx

  In our study, multivariate analysis showed that age, sex, marital status, grade, historical stage, surgery, site, and tumor size were independent prognostic factors for angiosarcoma. […] The C-index of the nomogram was significantly higher than that of the AJCC 6th edition and the AJCC 7th edition (0.74 vs 0.66 vs 0.61, respectively), which showed the accuracy and superiority of the nomogram.

• #31 Epidemiology of Angiosarcomas in Kentucky, 2000-2019

  https://uknowledge.uky.edu/cph_etds/388/

  Angiosarcomas are rare, highly aggressive malignant soft-tissue sarcomas of vascular or lymphatic origin. The purpose of this study is to describe geographical distribution of angiosarcoma in Kentucky and to examine the epidemiology of angiosarcoma in Kentucky from 2000 to 2019, including the most common sites where angiosarcomas occurred and risk factors affecting survival. […] The mean overall survival time observed in this sample was 50.2 months. Jefferson County had the highest case count (59), while Livingston County had the highest rate of angiosarcoma cases (21 per 100,000). The most common SEER sites among these cases were soft tissue, breast, and other non-epithelial skin. Patients older than 80 years old have significantly poorer overall survival than the other age groups, patients with angiosarcoma of the liver, lung and bronchus, and miscellaneous malignancies have a significant increased risk of death compared to other SEER sites, and there is a significant difference between the probabilities of death and SEER site, as well as insurance type.

• #32 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. Data of non-metastatic AS patients diagnosed after surgery between 2010 and 2015 was retrieved from the surveillance epidemiology and end results database. […] The median overall survival (OS) varied from 30 to 50 months for AS patients, resulting in a poor prognosis. […] Therefore, a more integrated system combining multi-factors is needed. […] However, limited data on this issue in non-metastatic AS patients after surgery has been published. […] Hence, by utilizing the data from surveillance epidemiology and end results (SEER) database, we present this study to predict the independent factors in non-metastatic AS after surgery and to construct a prognostic model for better guiding the clinical treatment and follow-up schedules for patients.

• #33 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

  The predictive accuracy of the nomograms was assessed by exhibiting C-index. The C-indexes were 0.757 (95%CI 0.6970.817) and 0.762 (95%CI 0.7020.822) for OS and CSS subjecting to the internal validation. In the external validation, the C-index for the OS nomogram was 0.749 (95%CI 0.6680.830), while for the CSS nomogram 0.756 (95%CI 0.6760.836). […] The results of the nomograms arrived at favorable consistency between predictive survival probabilities and the actual conditions, indicating that this model enabled us to distinguish hazard indicators and predict prognosis precisely.

• #34 Adrenal Epithelioid Angiosarcoma as a Rare Etiology of Chronic Abdominal Pain | ACS

  https://www.facs.org/for-medical-professionals/news-publications/journals/case-reviews/issues/v4n5/09-mesleh-adrenal-angiosarcoma/

  Surveillance is necessary, given the risk of local recurrence after retroperitoneal sarcoma resection. […] The patient in our case study will continue with this surveillance plan (physical exam, abdominal CT scan, and chest radiograph every six months for the upcoming years). […] Adrenal angiosarcoma is a rare tumor that requires a definitive diagnosis with histomorphology and immunohistochemistry. Treatment is surgical resection, with adjuvant radiation and chemotherapy in cases of positive margins and metastases, respectively.

• #35 Second Primary Malignancies After Initial Cutaneous Angiosarcoma: A SEER population-Based Study | SKIN The Journal of Cutaneous Medicine

  https://skin.dermsquared.com/skin/article/view/1498

  The epidemiology of second primary malignancies is an under-investigated domain within dermatology. […] While general epidemiological characteristics and survival data of patients with cutaneous angiosarcoma have been reported before, there is no investigation of the incidence and types of second primary malignancies (SPMs) that these patients face, which has relevance to screening and surveillance. […] Compared to a matched cohort from the general population, patients with CAS demonstrated increased incidence of new malignancies (SIR 1.54; 95% CI, 1.05-2.17; EAR 107.02). […] Importantly, CAS is associated with high recurrence even after complete resection.

• #36 Incidence and outcomes of cutaneous angiosarcoma: A SEER population-based study – EM consulte

  https://www.em-consulte.com/article/1382208/incidence-and-outcomes-of-cutaneous-angiosarcoma-a

  Cutaneous angiosarcoma (CAS) is a rare, malignant tumor of vascular mesenchymal origin accounting for less than 1% of all sarcomas. […] To examine epidemiologic trends and outcomes in CAS. […] In this retrospective, population-based study, patients with CAS were identified from the Surveillance Epidemiology and End Results database. Age, sex, and race-standardized incidence rates (IRs) were calculated. […] Of 811 patients with CAS, 43% had a prior primary cancer. CAS IR for patients without prior primary cancers dropped from 5.88 per 100,000 in 1973 to 1984 to 2.87 per 100,000 in 2005 to 2014. In those with prior primary cancers, IR rose from 0.03 per 100,000 in 1973 to 1984 to 2.25 per 100,000 in 2005 to 2014. […] CAS rates are rising among those with other prior primary cancers.

• #37

  https://typeset.io/papers/breast-angiosarcoma-surveillance-study-uk-national-audit-of-149fl215ie

  A detrimental impact of secondary angiosarcoma on breast cancer survival has been demonstrated and the increased use of adjuvant radiotherapy to treat low-risk breast cancer and DCIS is a cause for concern and warrants further study. […] Angiosarcomas have high recurrence and poor survival rates. […] This is concerning owing to the increasing use of adjuvant radiotherapy for the treatment of invasive breast cancer and ductal cancer in situ (DCIS), which could explain the rising incidence of angiosarcoma. […] Rates of recurrence were high with 14 of 28 (50 per cent) recurrences in patients with primary and 80 of 124 (64.5 per cent) in those with secondary angiosarcoma at 5 years. […] Development of secondary angiosarcoma had a negative impact on predicted breast cancer survival, with a median 10-year PREDICT prognostic rate of 69.6 per cent, compared with 54.0 per cent in the observed cohort. […] The increased use of adjuvant radiotherapy to treat low-risk breast cancer and DCIS is a cause for concern and warrants further study.

• #38 Angiosarcoma: a review of diagnosis and current treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6895451/

  The diagnosis of angiosarcoma remains a challenge. Due to its non-specificity of symptoms, it is difficult to discern angiosarcoma from other malignant neoplasms like anaplastic melanoma and epithelial carcinomas etc. […] The roles of ultrasound, computed tomography (CT) and magnetic resonance Imaging (MRI) in diagnosing angiosarcoma have their limitations. Therefore, histological examination is significant for the diagnosis of angiosarcomas and immunohistochemical confirmation is required. […] Delayed diagnosis and the rarity of these tumors contribute to the difficulties in regarding best treatment and prognostic factors, radical surgery followed by adjuvant radiotherapy is thought the current optimal modality. […] Regardless the controversy with respect to its side effect, conventional cytotoxic chemotherapy has been frequently used in treating inoperable and metastatic tumors. […] In addition, targeted medicines and immunotherapy have recently been studied as promising treatment for angiosarcomas.

• #39 MedicalResearch.com | Epidemiology and Outcomes of Angiosarcoma

  https://medicalresearch.com/epidemiology-and-outcomes-of-angiosarcoma/

  27 Dec Epidemiology and Outcomes of Angiosarcoma […] Angiosarcoma is an uncommon form of aggressive soft tissue sarcoma (STS). It comprises of 2 distinct subgroups of patients: one originating from the skin typically scalp, seen predominantly in elderly patients while the second subgroup affects younger patients and tends to originate from visceral organs including the liver and breasts. Angiosarcoma may also develop as a complication of prior radiation treatment. Prognosis in patient is poor, with survival generally less than 1 year in those with advanced or unresectable disease. Optimal treatment remains unclear; most published series on angiosarcoma tends to be small singlecenter studies. […] In our previous Asian STAR study evaluating epidemiology, treatment patterns and outcomes in Asian patients diagnosed with STS, we found that angiosarcoma represented 7% of the cohort, a proportion higher than what we would have expected from published series coming out of the west and hence our interest in this subject.

• #40 Incidence and site specific characteristics of angiosarcoma in Japan using a population-based national cancer registry from 2016 to 2019 | Scientific Reports

  https://www.nature.com/articles/s41598-025-94956-5

  The present study clarifies the epidemiology, clinical features, treatment, and prognosis of patients with angiosarcoma in Japan based on the NCR. This study is the first to have characterized the profiles of angiosarcoma on a national basis in a population-based manner in Japan and demonstrates disparities between Japan and Western countries, especially in age-adjusted incidence and survival.

• #41 MedicalResearch.com | Epidemiology and Outcomes of Angiosarcoma

  https://medicalresearch.com/epidemiology-and-outcomes-of-angiosarcoma/

  Prospective studies evaluating the role of pre-operative treatment, be it chemotherapy and/or radiation treatment, would be very important to move this field forward and help us optimize treatment in this subset of patients with localized angiosarcoma. […] Additionally, we would be very keen to understand the reasons behind the low usage of systemic treatment in patients with advanced angiosarcoma and act on those information in a systemic fashion to improve clinical outcomes for our patients. […] To our knowledge, this study by the Asian Sarcoma Consortium (ASC) comprising of 423 patients is one of the largest, if not largest, of its kind in angiosarcoma ever reported. It provides us with a first glimpse of our patients from Asia and serves as a basis for us to review our clinical practice so as to improve outcomes for our patients. Sarcoma being an uncommon disease would clearly benefit from collaborative research such as this in order to move the field forward.

• #42 MedicalResearch.com | Epidemiology and Outcomes of Angiosarcoma

  https://medicalresearch.com/epidemiology-and-outcomes-of-angiosarcoma/

  The data raises several provocative hypotheses. […] Firstly, the study highlights the chemo-sensitive nature of angiosarcoma and potential benefits of systemic treatment in this disease subset. Thus, in well selected patients, palliative systemic treatment should be considered. However, the reasons behind the relatively low use of systemic chemotherapy in the real world treatment of these patients is as yet unclear and would be an interesting subject of further investigation. […] In the localized setting, our data showed that a negative surgical margin was only achieved in approximately 70% of cases, and negative surgical margins had a direct impact on relapse-free survival. Thus our data calls for a re-examination of our treatment paradigm, arguing for the consideration of pre-operative therapies in bid to improve tumour resectability and clinical outcomes.

• #43 Angiosarcoma | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/angiosarcoma

  As physician scientists learn more about the genetic underpinnings of angiosarcoma, and seek to develop drugs that target specific mutations, the hope is the prognosis will improve. […] Much of our current angiosarcoma research is focused on trying to better understand the genetic underpinnings of angiosarcoma. […] For many children with rare or hard-to-treat conditions such as angiosarcoma, clinical trials provide new options.

• #44 Angiosarcoma | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/angiosarcoma

  Angiosarcoma is very rare — occurring in only about two people per million. […] All angiosarcomas need aggressive evaluation and treatment. […] Angiosarcoma is a type of vascular tumor. […] It is one of the most difficult types of vascular tumor to treat. […] The tumor behaves differently in each person. […] All angiosarcomas need aggressive evaluation and treatment. […] Because angiosarcoma is so rare, very few doctors have experience diagnosing and treating it. […] Our physicians — representing 16 medical and surgical specialties, including radiologists and pathologists who specialize in diagnosing vascular anomalies — take an interdisciplinary approach to care with every child we see. […] Our team takes an interdisciplinary approach to care with every child we see. […] The prognosis for children with localized (stage 1) angiosarcoma that can be completely surgically removed is good. However, for children with advanced angiosarcoma, the prognosis remains poor.

• #45 Angiosarcoma: Symptoms, Types & Treatment

  https://my.clevelandclinic.org/health/diseases/22778-angiosarcoma

  Angiosarcomas are rare malignant soft tissue sarcomas, which are rare tumors in and of themselves. Each year, approximately 1 person in 1 million people in the U.S. is diagnosed with angiosarcoma. The most common site where you get angiosarcoma is your skin, especially your scalp. […] Cancer staging helps healthcare providers classify how advanced cancer is. Providers stage angiosarcomas on a scale from I to IV, with Stage I meaning the tumor hasnt spread beyond its primary location and Stage IV meaning its spread to distant parts of the body. As angiosarcomas are aggressive, many have already spread (which means theyre more advanced) by the time theyre diagnosed. The most common place they spread is to the lungs. […] Knowing your cancer stage can help your provider decide which treatments may help you live longer. […] Angiosarcoma is a very rare cancer that spreads fast. This means that if you have angiosarcoma, you may be dealing with advanced cancer.

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