Sarcoma nabłonkowate
Rokowania, prognozy i postęp choroby

Mięsak nabłonkowaty (epithelioid sarcoma, ES) to rzadki, złośliwy nowotwór mezenchymalny o niekorzystnym rokowaniu, charakteryzujący się wysokim odsetkiem nawrotów miejscowych (34-77%) oraz przerzutów (około 40%), głównie do płuc, węzłów chłonnych, skóry, kości i mózgu. Mediana przeżycia całkowitego u pacjentów z przerzutowym ES wynosi około 12 miesięcy. Pięcioletnie przeżycie całkowite waha się od 25% do 70%, a specyficzne dla choroby wynosi około 55-68%, z tendencją stabilizacji krzywej przeżycia po około 100 miesiącach. Czynniki prognostyczne lepszego rokowania obejmują wiek poniżej 16 lat, lokalne stadium choroby (5-letnie przeżycie 75%), brak zajęcia węzłów chłonnych, wielkość guza ≤5 cm, dystalną i powierzchowną lokalizację guza, niski stopień złośliwości (G1-G2) oraz płeć żeńską. Z kolei gorsze rokowanie wiąże się z wiekiem >56 lat, chorobą regionalną lub przerzutową (0% 5-letniego przeżycia przy przerzutach odległych), obecnością przerzutów do węzłów chłonnych, guzem >5 cm, lokalizacją proksymalną i głęboką, wysokim stopniem złośliwości (G3-G4), męską płcią oraz dodatnimi marginesami chirurgicznymi lub brakiem operacji.

  1. Wprowadzenie do prognozy sarcoma nabłonkowatego
    1. Wskaźniki przeżycia
  2. Czynniki prognostyczne
    1. Wiek
    2. Stopień zaawansowania choroby
    3. Zajęcie węzłów chłonnych
    4. Wielkość i lokalizacja guza
    5. Stopień złośliwości
    6. Cechy histopatologiczne
    7. Płeć
  3. Wpływ leczenia na rokowanie
    1. Leczenie chirurgiczne
    2. Radioterapia
    3. Chemioterapia
    4. Terapie celowane
  4. Wnioski i zalecenia dotyczące monitorowania
    1. Kolejne rozdziały

Wprowadzenie do prognozy sarcoma nabłonkowatego

Sarcoma nabłonkowate (epithelioid sarcoma, ES) to rzadki, złośliwy nowotwór mezenchymalny, stanowiący mniej niż 1% mięsaków tkanek miękkich. Mimo powolnego wzrostu, ogólne rokowanie jest niekorzystne, z wysokim odsetkiem miejscowych nawrotów, przerzutów do węzłów chłonnych i przerzutów krwiopochodnych.1 Pacjenci z przerzutowym ES mają złe rokowanie z przewidywaną medianą przeżycia całkowitego wynoszącą około roku.2 Jednakże mięsak nabłonkowaty jest chorobą uleczalną, szczególnie gdy zostanie wykryty wcześnie.3

Wskaźniki przeżycia

Przeżycie pacjentów z ES jest niezadowalające. W zależności od badania, 5-letnie wskaźniki przeżycia całkowitego wahają się od 25% do 70%.4 Dane z bazy SEER wskazują na 5-letnie przeżycie specyficzne dla choroby wynoszące 68%, a 10-letnie na poziomie 61%. Krzywa przeżycia zaczyna się stabilizować po około 100 miesiącach, co sugeruje, że zgony występujące później można przypisać czynnikom innym niż ES.5 W innych badaniach raportowano 5-letnie przeżycie specyficzne dla choroby na poziomie 55,7%, 10-letni wskaźnik OS wynoszący 60,4%, częstość nawrotów 63,4% i częstość przerzutów 40,3%.6

U dzieci z mięsakiem nabłonkowatym rokowanie może być nieco lepsze niż u dorosłych, z 5-letnimi wskaźnikami przeżycia około 65%.7 Ogólny wskaźnik przeżycia 1-rocznego, 3-letniego i 5-letniego wynosi odpowiednio 70,7%, 56,1% i 50,4%.8

Czynniki prognostyczne

Rokowanie i przeżycie zależą od wielu czynników. Tylko lekarz znający historię medyczną pacjenta, typ i stadium choroby oraz inne cechy nowotworu, wybrane metody leczenia i odpowiedź na leczenie może połączyć wszystkie te informacje ze statystykami przeżycia, aby określić rokowanie.9

Wiek

Wiek jest istotnym czynnikiem prognostycznym w ES. W analizie wieloczynnikowej bazy SEER wykazano, że wiek poniżej 16 lat niezależnie prognozuje lepsze przeżycie specyficzne dla choroby.10 Osoby z mięsakiem tkanek miękkich w wieku poniżej 50 lat mają lepsze rokowanie niż osoby powyżej 50 roku życia.11 Pacjenci powyżej 56 roku życia są narażeni na wyższe ryzyko zgonu, jednak prognostyczne znaczenie wieku w ES jest nadal przedmiotem dyskusji.1213

Stopień zaawansowania choroby

Stopień zaawansowania choroby w momencie diagnozy jest krytycznym czynnikiem prognostycznym. Pacjenci z chorobą lokalną mają lepsze 5-letnie przeżycie wynoszące 75% w porównaniu z pacjentami z chorobą regionalną, u których 5-letnie przeżycie wynosi 49%.14 Żaden z pacjentów, u których w momencie diagnozy stwierdzono chorobę przerzutową, nie przeżył 5 lat, a 1-roczne przeżycie wynosiło 46%.15

Po pięciu latach od diagnozy pierwotnego guza, przerzuty stwierdza się u 30% do 75% pacjentów.16 Mięsak nabłonkowaty w IV stadium (przerzutowy mięsak tkanek miękkich) ma złe rokowanie.17

Zajęcie węzłów chłonnych

Przerzuty do węzłów chłonnych, choć rzadkie w większości mięsaków (występujące u mniej niż 3% pacjentów), są częstsze w niektórych podtypach, w tym w mięsaku nabłonkowatym.18 Zajęcie węzłów chłonnych zostało uznane za niezależny predyktor niekorzystnego rokowania.19 Obecność przerzutów do węzłów chłonnych w momencie pierwotnej diagnozy oraz obecność przerzutów odległych jest istotnie skorelowana z gorszym rokowaniem.20

W kontekście leczenia, nie należy lekceważyć przerzutów do węzłów chłonnych przed i po operacji. Guz powinien być usunięty razem z zajętym nerwem obwodowym. Ujemny margines chirurgiczny zgodnie z oceną śródoperacyjną jest niezbędny podczas operacji.21

Wielkość i lokalizacja guza

Wielkość guza tkanek miękkich jest ważnym czynnikiem prognostycznym. Guzy o średnicy 5 cm lub mniejsze mają lepsze rokowanie niż guzy większe niż 5 cm.22 Większy rozmiar pierwotnego guza ES w momencie diagnozy koreluje z krótszym przeżyciem całkowitym.23 Guzy o wielkości ponad 2 cm średnicy również korelują z gorszym rokowaniem.24

Lokalizacja guza również wpływa na rokowanie. Guzy tkanek miękkich znajdujące się blisko powierzchni ciała (guzy powierzchowne) mają lepsze rokowanie niż guzy rosnące głęboko w ciele. Guzy głęboko umiejscowione w tkance łącznej, która otacza mięśnie, nerwy, naczynia krwionośne i narządy (tzw. powięź), zwykle mają złe rokowanie.25

Mięsaki tkanek miękkich ramion, nóg lub powierzchni tułowia zwykle mają lepsze rokowanie niż mięsaki tkanek miękkich rozpoczynające się w innych częściach ciała.26 Lokalizacja proksymalna (bliższa do tułowia) wiąże się z gorszymi wynikami niż przypadki dystalne (odległe).27

Stopień złośliwości

Mięsaki o niskim stopniu złośliwości mają lepsze rokowanie, ponieważ są mniej prawdopodobne do dawania przerzutów lub nawrotów niż mięsaki o wysokim stopniu złośliwości.28 Guzy o wysokim stopniu złośliwości (stopnie 3 i 4) korelują z gorszymi wynikami, w tym z krótszym ogólnym przeżyciem niż stopień 2.29

Mięsaki o wyższym stopniu złośliwości są związane z wyższymi wskaźnikami niepowodzenia leczenia miejscowego i zwiększonym potencjałem przerzutowym.30 Wiek powyżej 56 lat, wyższy stopień złośliwości guza, wielkość guza powyżej 5 cm i obecność przerzutów są związane ze złym rokowaniem.31

Cechy histopatologiczne

Złe czynniki prognostyczne dla ES w literaturze obejmują proksymalną lub osiową lokalizację guza, zwiększoną wielkość i głębokość, krwotok, liczbę figur mitotycznych, martwicę, obecność inwazji naczyniowej oraz obecność przerzutów odległych w momencie diagnozy.32 Guzy z martwicą i inwazją naczyniową również korelują z gorszym rokowaniem.33

Płeć

Kobiety mają tendencję do lepszych wyników niż mężczyźni.34 Płeć żeńska w porównaniu z męską jest związana z bardziej pozytywnym rokowaniem.35

Wpływ leczenia na rokowanie

Leczenie chirurgiczne

Jedynym sposobem na wyleczenie ES jest całkowite usunięcie guza. Jednak całkowite usunięcie nie jest możliwe we wszystkich przypadkach.36 Pacjenci poddawani operacji mają lepsze wyniki niż pacjenci bez leczenia chirurgicznego (5-letnie przeżycie odpowiednio 68% i 33%).37

Mięsak tkanek miękkich, który zostaje całkowicie usunięty chirurgicznie, ma znacznie bardziej korzystne rokowanie niż mięsak tkanek miękkich, który nie zostaje całkowicie usunięty lub jeśli operacja nie jest możliwa.38

Jeśli komórki nowotworowe znajdują się w marginesie (tzw. dodatni margines chirurgiczny), rokowanie jest gorsze w porównaniu do sytuacji, gdy w marginesie nie ma komórek nowotworowych (tzw. ujemny margines chirurgiczny).39

Radioterapia

Istnieje potencjalna korzyść z radioterapii uzupełniającej w zmniejszaniu nawrotów po resekcji chirurgicznej, a radioterapia jest rutynowo stosowana jako uzupełnienie operacji.40 Resekcja chirurgiczna i limfadenektomia z pooperacyjną radioterapią lub bez niej mogą być wskazane u pacjentów z klinicznie dodatnimi węzłami chłonnymi.41

Mimo potencjalnych korzyści, w niektórych przypadkach guz może wykazywać agresywne zachowanie, z powiększającym się miejscowym nawrotem, przerzutami i rozsiewem w pozostałej części ośrodkowego układu nerwowego, nawet podczas radioterapii.42

Chemioterapia

Rola chemioterapii uzupełniającej w ES pozostaje niepewna w literaturze. Istniejące retrospektywne badania sugerują, że chemioterapia nie przynosi żadnych korzyści w zakresie przeżycia.43 Chemioterapia uzupełniająca może być rozważana, ale nie ma pewności, czy poprawia ogólne przeżycie.44

Mięsak nabłonkowaty jest oporny na konwencjonalną chemioterapię, z raportowanym średnim przeżyciem 52 tygodni przy chemioterapii paliatywnej.45 Ogólnie, korzyść z chemioterapii uzupełniającej w ES kręgosłupa pozostaje niejasna.46

Terapie celowane

Mięsak nabłonkowaty jest agresywnym STS, często opornym na chemioterapię i radioterapię, z ograniczonymi opcjami leczenia.47 Jednak nowsze terapie pokazują obiecujące wyniki:

  • Tazmetostat, inhibitor EZH2, został zatwierdzony w leczeniu zaawansowanego ES na podstawie badania klinicznego z udziałem 62 pacjentów, z ORR 14,5% (9/62), medianą PFS 5,5 miesiąca i medianą OS 19 miesięcy.48
  • SHR-2554, doustny selektywny inhibitor EZH2, w badaniu fazy 2 wykazał podobne wyniki do Tazmetostatu. Spośród 14 leczonych pacjentów z zaawansowanym ES, 3 uzyskało częściową odpowiedź.49
  • Pazopanib – w kohorcie 33 pacjentów leczonych pazopanibem zgłoszono dłuższą medianą PFS wynoszącą 10,2 miesiąca w porównaniu do badania PALETTE i innych opublikowanych badań dotyczących stosowania pazopanibu. Zaobserwowano ORR na poziomie 27%, przy medianie OS wynoszącej 17,8 miesiąca.50

Wnioski i zalecenia dotyczące monitorowania

Mięsak nabłonkowaty, mimo że jest nowotworem o powolnym wzroście, charakteryzuje się wysokim wskaźnikiem nawrotów wynoszącym od 34% do 77%, zależnie od adekwatności początkowego wycięcia.51 Przerzuty występują u 40% pacjentów, głównie do płuc, węzłów regionalnych, skóry głowy, kości i mózgu.52

Ze względu na wysokie ryzyko nawrotu i przerzutów, pacjenci wymagają ścisłej obserwacji, aby jak najwcześniej wykryć lokalny nawrót i/lub przerzuty do węzłów chłonnych.53 Wczesna diagnoza i leczenie są kluczowe dla lepszego rokowania.54

Podsumowując, niezależnymi czynnikami prognostycznymi lepszego przeżycia specyficznego dla choroby u pacjentów z ES są: wiek poniżej 16 lat, ujemne węzły chłonne lub miejscowe stadium choroby oraz możliwość operacyjnego leczenia choroby pierwotnej.55 Pomimo agresywnego charakteru, dzięki nowszym terapiom celowanym i dokładnemu monitorowaniu, wiele osób z tą chorobą może prowadzić długie, satysfakcjonujące życie.56

Czynnik prognostyczny Lepsze rokowanie Gorsze rokowanie
Wiek <16 lat, <50 lat >56 lat, >75 lat
Stadium choroby Miejscowe (5-letnie przeżycie 75%) Regionalne (5-letnie przeżycie 49%), odległe przerzuty (0% 5-letniego przeżycia)
Węzły chłonne Bez zajęcia Z przerzutami
Wielkość guza ≤5 cm >5 cm
Lokalizacja Dystalna, powierzchowna, kończyny Proksymalna, głęboka, osiowa
Stopień złośliwości Niski (G1-G2) Wysoki (G3-G4)
Płeć Żeńska Męska
Leczenie chirurgiczne Całkowite usunięcie, ujemne marginesy Niecałkowite usunięcie, dodatnie marginesy, brak operacji
Cechy histopatologiczne Brak martwicy, brak inwazji naczyniowej Martwica, inwazja naczyniowa, krwotok, wysoka aktywność mitotyczna

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report
    https://www.mdpi.com/2077-0383/12/17/5632
    Epithelioid sarcoma is a rare malignant mesenchymal tumor that represents less than 1% of soft-tissue sarcomas. Despite its slow growth, the overall prognosis is poor with a high rate of local recurrence, lymph-node spread, and hematogenous metastasis. […] The disease has a poor prognosis, with high propensity for local recurrence, as well as lymphatic and hematogenous metastatic spread. […] Recurrence is often noted to occur, with a recurrence rate varying from 34% and 77% depending on the adequacy of initial excision. […] Metastasis occurs in 40% of patients, predominantly involving the lungs, regional nodes, scalp, bone, and brain. […] Poor prognostic factors for ES in the literature include a proximal or axial tumor location, increased size and depth, hemorrhage, mitotic figures, necrosis, presence of vascular invasion, and the presence of distant metastases at the time of diagnosis.
  • #2 Epithelioid Sarcoma—From Genetics to Clinical Practice
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/
    Epithelioid sarcoma is known for aggressive behavior, including a high recurrence rate and regional lymph node metastases. […] Patients with metastatic ES have a poor prognosis with an expected median overall survival of about a year. […] The survival of patients with ES is unsatisfactory. Depending on the study, the 5-year overall survival rates from 25% to 70%. […] Five years on from primary tumor diagnosis, metastatic spread is found in 30% to 75% of patients. […] The reported 5-year disease-specific survival was 55.7%, the 10-year OS rate was 60.4%, the recurrence rate was 63.4%, and the metastasis rate was 40.3%. […] Patients older than 55 years are to be at a higher risk of death; however, age prognostic significance in ES is still under debate. […] The larger size of the ES primary tumor at primary diagnosis is correlated with shorter overall survival. […] High-grade tumors (grades 3 and 4) are correlated with worse outcomes, including shorter overall survival than grade 2. […] The presence of lymph nodes metastases at the primary diagnosis and the presence of distant metastases is significantly correlated with the poor prognosis.
  • #3 Epithelioid Sarcoma: Symptoms, Treatment & Prognosis
    https://my.clevelandclinic.org/health/diseases/24331-epithelioid-sarcoma
    Epithelioid sarcoma is treatable, especially when detected early. […] The only way to cure ES is to completely remove the tumor. But complete removal isn’t possible in all cases. […] Because ES is slow growing, many people with the disease can still lead long, fulfilling lives. […] The five-year survival rate for epithelioid sarcoma ranges from 25% to 92%, depending on which study you read.
  • #4 Epithelioid Sarcoma—From Genetics to Clinical Practice
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/
    Epithelioid sarcoma is known for aggressive behavior, including a high recurrence rate and regional lymph node metastases. […] Patients with metastatic ES have a poor prognosis with an expected median overall survival of about a year. […] The survival of patients with ES is unsatisfactory. Depending on the study, the 5-year overall survival rates from 25% to 70%. […] Five years on from primary tumor diagnosis, metastatic spread is found in 30% to 75% of patients. […] The reported 5-year disease-specific survival was 55.7%, the 10-year OS rate was 60.4%, the recurrence rate was 63.4%, and the metastasis rate was 40.3%. […] Patients older than 55 years are to be at a higher risk of death; however, age prognostic significance in ES is still under debate. […] The larger size of the ES primary tumor at primary diagnosis is correlated with shorter overall survival. […] High-grade tumors (grades 3 and 4) are correlated with worse outcomes, including shorter overall survival than grade 2. […] The presence of lymph nodes metastases at the primary diagnosis and the presence of distant metastases is significantly correlated with the poor prognosis.
  • #5 Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database
    https://pmc.ncbi.nlm.nih.gov/articles/PMC2758965/
    Current stratification of prognosis in patients with epithelioid sarcoma (ES) is based largely on data reported by individual centers with a limited number of patients. […] On multivariate analysis, only age younger than 16 years, local stage of disease, or negative nodes and surgical resection of the tumor predicted better disease-specific survival. […] The SEER database shows only age younger than 16 years, negative nodes, or local stage of disease and operability of primary disease independently predict survival in patients with ES. […] The disease-specific survival was 68% at 5 years and 61% at 10 years, and started to plateau at approximately 100 months, which suggests deaths occurring thereafter can be attributed to factors other than ES. […] Patients diagnosed with local disease had a better 5-year survival of 75% as compared with patients with regional disease who had a 5-year survival of 49%.
  • #6 Epithelioid Sarcoma—From Genetics to Clinical Practice
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/
    Epithelioid sarcoma is known for aggressive behavior, including a high recurrence rate and regional lymph node metastases. […] Patients with metastatic ES have a poor prognosis with an expected median overall survival of about a year. […] The survival of patients with ES is unsatisfactory. Depending on the study, the 5-year overall survival rates from 25% to 70%. […] Five years on from primary tumor diagnosis, metastatic spread is found in 30% to 75% of patients. […] The reported 5-year disease-specific survival was 55.7%, the 10-year OS rate was 60.4%, the recurrence rate was 63.4%, and the metastasis rate was 40.3%. […] Patients older than 55 years are to be at a higher risk of death; however, age prognostic significance in ES is still under debate. […] The larger size of the ES primary tumor at primary diagnosis is correlated with shorter overall survival. […] High-grade tumors (grades 3 and 4) are correlated with worse outcomes, including shorter overall survival than grade 2. […] The presence of lymph nodes metastases at the primary diagnosis and the presence of distant metastases is significantly correlated with the poor prognosis.
  • #7 Epithelioid sarcoma – Wikipedia
    https://en.wikipedia.org/wiki/Epithelioid_sarcoma
    The 5-year survival rate for epithelioid sarcoma patients is usually quoted as 50-70%, with the 10-year survival rate is 42-55%. Children with epithelioid sarcoma may have somewhat better outcomes than adults, with 5 year survival rates around 65%. […] In addition to stage and grade of the tumor, gender, site, age at diagnosis, tumor size and microscopic pathology all have been shown to affect prognosis. […] Unsurprisingly, advanced stage and grade are associated with worse outcomes. Females tend to have more favorable outcomes than males, proximal cases show worse outcomes than distal cases. Tumors more than 2 cm in diameter and tumors with necrosis and vascular invasion also have been correlated with a worse outcome.
  • #8 The diagnosis and treatment of the epithelioid sarcomas involving the peripheral nerves | Scientific Reports
    https://www.nature.com/articles/s41598-024-82357-z
    Epithelioid sarcomas are rare soft tissue tumors and have possibility to involve the peripheral nerve and present as sensory and motor disorders. […] Early diagnosis and treatment are the keys to better prognosis. […] The symptoms presented were similar to those of the nerve compression diseases, which usually led to misdiagnosis and mistreatment. Early diagnosis and early treatment are the keys to better prognosis. […] The overall 1-year, 3-year and 5-year survival were 70.7, 56.1 and 50.4%, respectively. The overall recurrence and metastasis rates were 63.4% and 40.3%. Age more than 56 years old, higher tumor grade, tumor size over 5 cm and the presence of metastasis are related to poor prognosis. […] We should not ignore the lymph node metastasis pre- and postoperatively. The tumor should be resected together with the peripheral nerve involved. A negative surgical margin according to the frozen pathology is essential during the operation. […] Postoperative chemotherapy and radiotherapy are still controversial in the literature. The patients need close follow-up to discovered the local recurrence and/or lymph node metastasis as early as possible.
  • #9 Prognosis and survival for soft tissue sarcoma | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/soft-tissue-sarcoma/prognosis-and-survival
    If you have soft tissue sarcoma, you may have questions about your prognosis. A prognosis is the doctors best estimate of how cancer will affect someone and how it will respond to treatment. Prognosis and survival depend on many factors. Only a doctor familiar with your medical history, the type and stage and other features of the cancer, the treatments chosen and the response to treatment can put all of this information together with survival statistics to arrive at a prognosis. […] The following are prognostic and predictive factors for soft tissue sarcoma. […] The size of a soft tissue tumour is an important prognostic factor. Tumours that are 5 cm or smaller have a better prognosis than tumours larger than 5 cm. […] Low-grade soft tissue sarcomas are linked with a better prognosis because they are less likely to spread (metastasize) or come back (recur) than high-grade sarcomas.
  • #10 Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database
    https://pmc.ncbi.nlm.nih.gov/articles/PMC2758965/
    Current stratification of prognosis in patients with epithelioid sarcoma (ES) is based largely on data reported by individual centers with a limited number of patients. […] On multivariate analysis, only age younger than 16 years, local stage of disease, or negative nodes and surgical resection of the tumor predicted better disease-specific survival. […] The SEER database shows only age younger than 16 years, negative nodes, or local stage of disease and operability of primary disease independently predict survival in patients with ES. […] The disease-specific survival was 68% at 5 years and 61% at 10 years, and started to plateau at approximately 100 months, which suggests deaths occurring thereafter can be attributed to factors other than ES. […] Patients diagnosed with local disease had a better 5-year survival of 75% as compared with patients with regional disease who had a 5-year survival of 49%.
  • #11 Prognosis and survival for soft tissue sarcoma | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/soft-tissue-sarcoma/prognosis-and-survival
    If cancer cells are found in the margin (called a positive surgical margin), there is a poorer prognosis compared to when there are no cancer cells in the margin (called a negative surgical margin). […] Soft tissue sarcoma that is completely removed by surgery has a much more favourable prognosis than soft tissue sarcoma that is not completely removed or if surgery cant be done. […] People with soft tissue sarcoma who are under the age of 50 tend to have a better prognosis than those over the age of 50.
  • #12 Epithelioid Sarcoma—From Genetics to Clinical Practice
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/
    Epithelioid sarcoma is known for aggressive behavior, including a high recurrence rate and regional lymph node metastases. […] Patients with metastatic ES have a poor prognosis with an expected median overall survival of about a year. […] The survival of patients with ES is unsatisfactory. Depending on the study, the 5-year overall survival rates from 25% to 70%. […] Five years on from primary tumor diagnosis, metastatic spread is found in 30% to 75% of patients. […] The reported 5-year disease-specific survival was 55.7%, the 10-year OS rate was 60.4%, the recurrence rate was 63.4%, and the metastasis rate was 40.3%. […] Patients older than 55 years are to be at a higher risk of death; however, age prognostic significance in ES is still under debate. […] The larger size of the ES primary tumor at primary diagnosis is correlated with shorter overall survival. […] High-grade tumors (grades 3 and 4) are correlated with worse outcomes, including shorter overall survival than grade 2. […] The presence of lymph nodes metastases at the primary diagnosis and the presence of distant metastases is significantly correlated with the poor prognosis.
  • #13 The diagnosis and treatment of the epithelioid sarcomas involving the peripheral nerves | Scientific Reports
    https://www.nature.com/articles/s41598-024-82357-z
    Epithelioid sarcomas are rare soft tissue tumors and have possibility to involve the peripheral nerve and present as sensory and motor disorders. […] Early diagnosis and treatment are the keys to better prognosis. […] The symptoms presented were similar to those of the nerve compression diseases, which usually led to misdiagnosis and mistreatment. Early diagnosis and early treatment are the keys to better prognosis. […] The overall 1-year, 3-year and 5-year survival were 70.7, 56.1 and 50.4%, respectively. The overall recurrence and metastasis rates were 63.4% and 40.3%. Age more than 56 years old, higher tumor grade, tumor size over 5 cm and the presence of metastasis are related to poor prognosis. […] We should not ignore the lymph node metastasis pre- and postoperatively. The tumor should be resected together with the peripheral nerve involved. A negative surgical margin according to the frozen pathology is essential during the operation. […] Postoperative chemotherapy and radiotherapy are still controversial in the literature. The patients need close follow-up to discovered the local recurrence and/or lymph node metastasis as early as possible.
  • #14 Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database
    https://pmc.ncbi.nlm.nih.gov/articles/PMC2758965/
    Current stratification of prognosis in patients with epithelioid sarcoma (ES) is based largely on data reported by individual centers with a limited number of patients. […] On multivariate analysis, only age younger than 16 years, local stage of disease, or negative nodes and surgical resection of the tumor predicted better disease-specific survival. […] The SEER database shows only age younger than 16 years, negative nodes, or local stage of disease and operability of primary disease independently predict survival in patients with ES. […] The disease-specific survival was 68% at 5 years and 61% at 10 years, and started to plateau at approximately 100 months, which suggests deaths occurring thereafter can be attributed to factors other than ES. […] Patients diagnosed with local disease had a better 5-year survival of 75% as compared with patients with regional disease who had a 5-year survival of 49%.
  • #15 Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database
    https://pmc.ncbi.nlm.nih.gov/articles/PMC2758965/
    None of the patients who presented with distant disease survived for 5 years, and 1-year survival was 46%. […] Patients undergoing surgery fared better than patients with no surgical treatment (5-year survival 68% and 33%, respectively). […] Among independent demographic variables, age older than 75 years, distant disease, and absence of surgical resection independently predicted lower overall survival. […] Nodal metastasis has been recognized as an independent predictor of adverse prognosis, with a notable exception in a series reported by Ross et al. […] We report nodal involvement of regional and/or distant disease as an independent prognostic factor for adverse outcomes. […] Our study showed a 5-year disease-specific survival (68%) in this range.
  • #16 Epithelioid Sarcoma—From Genetics to Clinical Practice
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/
    Epithelioid sarcoma is known for aggressive behavior, including a high recurrence rate and regional lymph node metastases. […] Patients with metastatic ES have a poor prognosis with an expected median overall survival of about a year. […] The survival of patients with ES is unsatisfactory. Depending on the study, the 5-year overall survival rates from 25% to 70%. […] Five years on from primary tumor diagnosis, metastatic spread is found in 30% to 75% of patients. […] The reported 5-year disease-specific survival was 55.7%, the 10-year OS rate was 60.4%, the recurrence rate was 63.4%, and the metastasis rate was 40.3%. […] Patients older than 55 years are to be at a higher risk of death; however, age prognostic significance in ES is still under debate. […] The larger size of the ES primary tumor at primary diagnosis is correlated with shorter overall survival. […] High-grade tumors (grades 3 and 4) are correlated with worse outcomes, including shorter overall survival than grade 2. […] The presence of lymph nodes metastases at the primary diagnosis and the presence of distant metastases is significantly correlated with the poor prognosis.
  • #17 Prognosis and survival for soft tissue sarcoma | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/soft-tissue-sarcoma/prognosis-and-survival
    Soft tissue tumours close to the surface of the body (superficial tumours) have a better prognosis than tumours that are growing deep within the body. Tumours that are deep within the connective tissue that wraps around muscles, nerves, blood vessels and organs (called fascia) tend to have a poor prognosis. […] Soft tissue sarcomas of the arms, legs or surface of the trunk of the body usually have a better prognosis than soft tissue sarcomas that start in other parts of the body. […] Some types of soft tissue sarcoma tend to have a worse prognosis, including malignant peripheral nerve sheath tumours (MPNSTs), leiomyosarcoma, desmoplastic small round cell tumours and epithelioid sarcoma. […] The lower the stage of soft tissue sarcoma at diagnosis, the better the prognosis. Stage 4 soft tissue sarcoma (metastatic soft tissue sarcoma) has a poor prognosis. Soft tissue sarcoma that has grown into bone, nerves or vessels usually has a poor prognosis.
  • #18 Soft Tissue Sarcoma Treatment (PDQ®) – NCI
    https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq
    Prognostic nomograms (incorporating specific variables) have been developed for soft tissue sarcomas of the retroperitoneum and the extremities.[11-14] […] Poor prognostic factors in adults with soft tissue sarcomas include:[4-15] […] Higher-grade sarcomas are associated with higher local-treatment failure rates and increased metastatic potential.[16] […] Knowledge of intracompartmental or extracompartmental extension of extremity sarcomas is important for surgical decision making. […] Nodal involvement is rare, occurring in less than 3% of patients with sarcoma, but it occurs more often in certain subtypes, such as rhabdomyosarcoma, angiosarcoma, synovial sarcoma, clear cell sarcoma, and epithelioid sarcoma.[7,8] […] The prognosis for patients with high-grade retroperitoneal sarcomas is less favorable than for patients with tumors at other sites, partly because of the difficulty in completely resecting these tumors and the dose-limiting toxicity of high-dose radiation therapy on visceral organs.[13-16]
  • #19 Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database
    https://pmc.ncbi.nlm.nih.gov/articles/PMC2758965/
    None of the patients who presented with distant disease survived for 5 years, and 1-year survival was 46%. […] Patients undergoing surgery fared better than patients with no surgical treatment (5-year survival 68% and 33%, respectively). […] Among independent demographic variables, age older than 75 years, distant disease, and absence of surgical resection independently predicted lower overall survival. […] Nodal metastasis has been recognized as an independent predictor of adverse prognosis, with a notable exception in a series reported by Ross et al. […] We report nodal involvement of regional and/or distant disease as an independent prognostic factor for adverse outcomes. […] Our study showed a 5-year disease-specific survival (68%) in this range.
  • #20 Epithelioid Sarcoma—From Genetics to Clinical Practice
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/
    Epithelioid sarcoma is known for aggressive behavior, including a high recurrence rate and regional lymph node metastases. […] Patients with metastatic ES have a poor prognosis with an expected median overall survival of about a year. […] The survival of patients with ES is unsatisfactory. Depending on the study, the 5-year overall survival rates from 25% to 70%. […] Five years on from primary tumor diagnosis, metastatic spread is found in 30% to 75% of patients. […] The reported 5-year disease-specific survival was 55.7%, the 10-year OS rate was 60.4%, the recurrence rate was 63.4%, and the metastasis rate was 40.3%. […] Patients older than 55 years are to be at a higher risk of death; however, age prognostic significance in ES is still under debate. […] The larger size of the ES primary tumor at primary diagnosis is correlated with shorter overall survival. […] High-grade tumors (grades 3 and 4) are correlated with worse outcomes, including shorter overall survival than grade 2. […] The presence of lymph nodes metastases at the primary diagnosis and the presence of distant metastases is significantly correlated with the poor prognosis.
  • #21 The diagnosis and treatment of the epithelioid sarcomas involving the peripheral nerves | Scientific Reports
    https://www.nature.com/articles/s41598-024-82357-z
    Epithelioid sarcomas are rare soft tissue tumors and have possibility to involve the peripheral nerve and present as sensory and motor disorders. […] Early diagnosis and treatment are the keys to better prognosis. […] The symptoms presented were similar to those of the nerve compression diseases, which usually led to misdiagnosis and mistreatment. Early diagnosis and early treatment are the keys to better prognosis. […] The overall 1-year, 3-year and 5-year survival were 70.7, 56.1 and 50.4%, respectively. The overall recurrence and metastasis rates were 63.4% and 40.3%. Age more than 56 years old, higher tumor grade, tumor size over 5 cm and the presence of metastasis are related to poor prognosis. […] We should not ignore the lymph node metastasis pre- and postoperatively. The tumor should be resected together with the peripheral nerve involved. A negative surgical margin according to the frozen pathology is essential during the operation. […] Postoperative chemotherapy and radiotherapy are still controversial in the literature. The patients need close follow-up to discovered the local recurrence and/or lymph node metastasis as early as possible.
  • #22 Prognosis and survival for soft tissue sarcoma | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/soft-tissue-sarcoma/prognosis-and-survival
    If you have soft tissue sarcoma, you may have questions about your prognosis. A prognosis is the doctors best estimate of how cancer will affect someone and how it will respond to treatment. Prognosis and survival depend on many factors. Only a doctor familiar with your medical history, the type and stage and other features of the cancer, the treatments chosen and the response to treatment can put all of this information together with survival statistics to arrive at a prognosis. […] The following are prognostic and predictive factors for soft tissue sarcoma. […] The size of a soft tissue tumour is an important prognostic factor. Tumours that are 5 cm or smaller have a better prognosis than tumours larger than 5 cm. […] Low-grade soft tissue sarcomas are linked with a better prognosis because they are less likely to spread (metastasize) or come back (recur) than high-grade sarcomas.
  • #23 Epithelioid Sarcoma—From Genetics to Clinical Practice
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/
    Epithelioid sarcoma is known for aggressive behavior, including a high recurrence rate and regional lymph node metastases. […] Patients with metastatic ES have a poor prognosis with an expected median overall survival of about a year. […] The survival of patients with ES is unsatisfactory. Depending on the study, the 5-year overall survival rates from 25% to 70%. […] Five years on from primary tumor diagnosis, metastatic spread is found in 30% to 75% of patients. […] The reported 5-year disease-specific survival was 55.7%, the 10-year OS rate was 60.4%, the recurrence rate was 63.4%, and the metastasis rate was 40.3%. […] Patients older than 55 years are to be at a higher risk of death; however, age prognostic significance in ES is still under debate. […] The larger size of the ES primary tumor at primary diagnosis is correlated with shorter overall survival. […] High-grade tumors (grades 3 and 4) are correlated with worse outcomes, including shorter overall survival than grade 2. […] The presence of lymph nodes metastases at the primary diagnosis and the presence of distant metastases is significantly correlated with the poor prognosis.
  • #24 Epithelioid sarcoma – Wikipedia
    https://en.wikipedia.org/wiki/Epithelioid_sarcoma
    The 5-year survival rate for epithelioid sarcoma patients is usually quoted as 50-70%, with the 10-year survival rate is 42-55%. Children with epithelioid sarcoma may have somewhat better outcomes than adults, with 5 year survival rates around 65%. […] In addition to stage and grade of the tumor, gender, site, age at diagnosis, tumor size and microscopic pathology all have been shown to affect prognosis. […] Unsurprisingly, advanced stage and grade are associated with worse outcomes. Females tend to have more favorable outcomes than males, proximal cases show worse outcomes than distal cases. Tumors more than 2 cm in diameter and tumors with necrosis and vascular invasion also have been correlated with a worse outcome.
  • #25 Prognosis and survival for soft tissue sarcoma | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/soft-tissue-sarcoma/prognosis-and-survival
    Soft tissue tumours close to the surface of the body (superficial tumours) have a better prognosis than tumours that are growing deep within the body. Tumours that are deep within the connective tissue that wraps around muscles, nerves, blood vessels and organs (called fascia) tend to have a poor prognosis. […] Soft tissue sarcomas of the arms, legs or surface of the trunk of the body usually have a better prognosis than soft tissue sarcomas that start in other parts of the body. […] Some types of soft tissue sarcoma tend to have a worse prognosis, including malignant peripheral nerve sheath tumours (MPNSTs), leiomyosarcoma, desmoplastic small round cell tumours and epithelioid sarcoma. […] The lower the stage of soft tissue sarcoma at diagnosis, the better the prognosis. Stage 4 soft tissue sarcoma (metastatic soft tissue sarcoma) has a poor prognosis. Soft tissue sarcoma that has grown into bone, nerves or vessels usually has a poor prognosis.
  • #26 Prognosis and survival for soft tissue sarcoma | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/soft-tissue-sarcoma/prognosis-and-survival
    Soft tissue tumours close to the surface of the body (superficial tumours) have a better prognosis than tumours that are growing deep within the body. Tumours that are deep within the connective tissue that wraps around muscles, nerves, blood vessels and organs (called fascia) tend to have a poor prognosis. […] Soft tissue sarcomas of the arms, legs or surface of the trunk of the body usually have a better prognosis than soft tissue sarcomas that start in other parts of the body. […] Some types of soft tissue sarcoma tend to have a worse prognosis, including malignant peripheral nerve sheath tumours (MPNSTs), leiomyosarcoma, desmoplastic small round cell tumours and epithelioid sarcoma. […] The lower the stage of soft tissue sarcoma at diagnosis, the better the prognosis. Stage 4 soft tissue sarcoma (metastatic soft tissue sarcoma) has a poor prognosis. Soft tissue sarcoma that has grown into bone, nerves or vessels usually has a poor prognosis.
  • #27 Epithelioid sarcoma – Wikipedia
    https://en.wikipedia.org/wiki/Epithelioid_sarcoma
    The 5-year survival rate for epithelioid sarcoma patients is usually quoted as 50-70%, with the 10-year survival rate is 42-55%. Children with epithelioid sarcoma may have somewhat better outcomes than adults, with 5 year survival rates around 65%. […] In addition to stage and grade of the tumor, gender, site, age at diagnosis, tumor size and microscopic pathology all have been shown to affect prognosis. […] Unsurprisingly, advanced stage and grade are associated with worse outcomes. Females tend to have more favorable outcomes than males, proximal cases show worse outcomes than distal cases. Tumors more than 2 cm in diameter and tumors with necrosis and vascular invasion also have been correlated with a worse outcome.
  • #28 Prognosis and survival for soft tissue sarcoma | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/soft-tissue-sarcoma/prognosis-and-survival
    If you have soft tissue sarcoma, you may have questions about your prognosis. A prognosis is the doctors best estimate of how cancer will affect someone and how it will respond to treatment. Prognosis and survival depend on many factors. Only a doctor familiar with your medical history, the type and stage and other features of the cancer, the treatments chosen and the response to treatment can put all of this information together with survival statistics to arrive at a prognosis. […] The following are prognostic and predictive factors for soft tissue sarcoma. […] The size of a soft tissue tumour is an important prognostic factor. Tumours that are 5 cm or smaller have a better prognosis than tumours larger than 5 cm. […] Low-grade soft tissue sarcomas are linked with a better prognosis because they are less likely to spread (metastasize) or come back (recur) than high-grade sarcomas.
  • #29 Epithelioid Sarcoma—From Genetics to Clinical Practice
    https://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/
    Epithelioid sarcoma is known for aggressive behavior, including a high recurrence rate and regional lymph node metastases. […] Patients with metastatic ES have a poor prognosis with an expected median overall survival of about a year. […] The survival of patients with ES is unsatisfactory. Depending on the study, the 5-year overall survival rates from 25% to 70%. […] Five years on from primary tumor diagnosis, metastatic spread is found in 30% to 75% of patients. […] The reported 5-year disease-specific survival was 55.7%, the 10-year OS rate was 60.4%, the recurrence rate was 63.4%, and the metastasis rate was 40.3%. […] Patients older than 55 years are to be at a higher risk of death; however, age prognostic significance in ES is still under debate. […] The larger size of the ES primary tumor at primary diagnosis is correlated with shorter overall survival. […] High-grade tumors (grades 3 and 4) are correlated with worse outcomes, including shorter overall survival than grade 2. […] The presence of lymph nodes metastases at the primary diagnosis and the presence of distant metastases is significantly correlated with the poor prognosis.
  • #30 Soft Tissue Sarcoma Treatment (PDQ®) – NCI
    https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq
    Prognostic nomograms (incorporating specific variables) have been developed for soft tissue sarcomas of the retroperitoneum and the extremities.[11-14] […] Poor prognostic factors in adults with soft tissue sarcomas include:[4-15] […] Higher-grade sarcomas are associated with higher local-treatment failure rates and increased metastatic potential.[16] […] Knowledge of intracompartmental or extracompartmental extension of extremity sarcomas is important for surgical decision making. […] Nodal involvement is rare, occurring in less than 3% of patients with sarcoma, but it occurs more often in certain subtypes, such as rhabdomyosarcoma, angiosarcoma, synovial sarcoma, clear cell sarcoma, and epithelioid sarcoma.[7,8] […] The prognosis for patients with high-grade retroperitoneal sarcomas is less favorable than for patients with tumors at other sites, partly because of the difficulty in completely resecting these tumors and the dose-limiting toxicity of high-dose radiation therapy on visceral organs.[13-16]
  • #31 The diagnosis and treatment of the epithelioid sarcomas involving the peripheral nerves | Scientific Reports
    https://www.nature.com/articles/s41598-024-82357-z
    Epithelioid sarcomas are rare soft tissue tumors and have possibility to involve the peripheral nerve and present as sensory and motor disorders. […] Early diagnosis and treatment are the keys to better prognosis. […] The symptoms presented were similar to those of the nerve compression diseases, which usually led to misdiagnosis and mistreatment. Early diagnosis and early treatment are the keys to better prognosis. […] The overall 1-year, 3-year and 5-year survival were 70.7, 56.1 and 50.4%, respectively. The overall recurrence and metastasis rates were 63.4% and 40.3%. Age more than 56 years old, higher tumor grade, tumor size over 5 cm and the presence of metastasis are related to poor prognosis. […] We should not ignore the lymph node metastasis pre- and postoperatively. The tumor should be resected together with the peripheral nerve involved. A negative surgical margin according to the frozen pathology is essential during the operation. […] Postoperative chemotherapy and radiotherapy are still controversial in the literature. The patients need close follow-up to discovered the local recurrence and/or lymph node metastasis as early as possible.
  • #32 Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report
    https://www.mdpi.com/2077-0383/12/17/5632
    Epithelioid sarcoma is a rare malignant mesenchymal tumor that represents less than 1% of soft-tissue sarcomas. Despite its slow growth, the overall prognosis is poor with a high rate of local recurrence, lymph-node spread, and hematogenous metastasis. […] The disease has a poor prognosis, with high propensity for local recurrence, as well as lymphatic and hematogenous metastatic spread. […] Recurrence is often noted to occur, with a recurrence rate varying from 34% and 77% depending on the adequacy of initial excision. […] Metastasis occurs in 40% of patients, predominantly involving the lungs, regional nodes, scalp, bone, and brain. […] Poor prognostic factors for ES in the literature include a proximal or axial tumor location, increased size and depth, hemorrhage, mitotic figures, necrosis, presence of vascular invasion, and the presence of distant metastases at the time of diagnosis.
  • #33 Epithelioid sarcoma – Wikipedia
    https://en.wikipedia.org/wiki/Epithelioid_sarcoma
    The 5-year survival rate for epithelioid sarcoma patients is usually quoted as 50-70%, with the 10-year survival rate is 42-55%. Children with epithelioid sarcoma may have somewhat better outcomes than adults, with 5 year survival rates around 65%. […] In addition to stage and grade of the tumor, gender, site, age at diagnosis, tumor size and microscopic pathology all have been shown to affect prognosis. […] Unsurprisingly, advanced stage and grade are associated with worse outcomes. Females tend to have more favorable outcomes than males, proximal cases show worse outcomes than distal cases. Tumors more than 2 cm in diameter and tumors with necrosis and vascular invasion also have been correlated with a worse outcome.
  • #34 Epithelioid sarcoma – Wikipedia
    https://en.wikipedia.org/wiki/Epithelioid_sarcoma
    The 5-year survival rate for epithelioid sarcoma patients is usually quoted as 50-70%, with the 10-year survival rate is 42-55%. Children with epithelioid sarcoma may have somewhat better outcomes than adults, with 5 year survival rates around 65%. […] In addition to stage and grade of the tumor, gender, site, age at diagnosis, tumor size and microscopic pathology all have been shown to affect prognosis. […] Unsurprisingly, advanced stage and grade are associated with worse outcomes. Females tend to have more favorable outcomes than males, proximal cases show worse outcomes than distal cases. Tumors more than 2 cm in diameter and tumors with necrosis and vascular invasion also have been correlated with a worse outcome.
  • #35 Epithelioid sarcoma – Wikipedia
    https://en.wikipedia.org/wiki/Epithelioid_sarcoma
    Epithelioid sarcoma most commonly strikes young adults, yet no age group is immune. The disease has a tendency to develop local recurrences and metastasis thereafter to regional lymph nodes, lung, bone, brain, and other locations. […] Generally speaking, epithelioid sarcoma has a high rate of relapse after initial treatment and tends to recur locally or regionally (at or near the original tumor site). Epithelioid sarcoma also demonstrates lymphatic spread (in 22-48% of cases), and metastasis (in 21-63% of cases). […] These events, as well as advanced stage (progression) and grade (aggressiveness), are predictive of an overall worse outcome. Associated with a more positive outcome are younger age, female vs. male sex, distal vs. proximal location, smaller tumor size, and negative margins upon tumor resection.
  • #36 Epithelioid Sarcoma: Symptoms, Treatment & Prognosis
    https://my.clevelandclinic.org/health/diseases/24331-epithelioid-sarcoma
    Epithelioid sarcoma is treatable, especially when detected early. […] The only way to cure ES is to completely remove the tumor. But complete removal isn’t possible in all cases. […] Because ES is slow growing, many people with the disease can still lead long, fulfilling lives. […] The five-year survival rate for epithelioid sarcoma ranges from 25% to 92%, depending on which study you read.
  • #37 Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database
    https://pmc.ncbi.nlm.nih.gov/articles/PMC2758965/
    None of the patients who presented with distant disease survived for 5 years, and 1-year survival was 46%. […] Patients undergoing surgery fared better than patients with no surgical treatment (5-year survival 68% and 33%, respectively). […] Among independent demographic variables, age older than 75 years, distant disease, and absence of surgical resection independently predicted lower overall survival. […] Nodal metastasis has been recognized as an independent predictor of adverse prognosis, with a notable exception in a series reported by Ross et al. […] We report nodal involvement of regional and/or distant disease as an independent prognostic factor for adverse outcomes. […] Our study showed a 5-year disease-specific survival (68%) in this range.
  • #38 Prognosis and survival for soft tissue sarcoma | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/soft-tissue-sarcoma/prognosis-and-survival
    If cancer cells are found in the margin (called a positive surgical margin), there is a poorer prognosis compared to when there are no cancer cells in the margin (called a negative surgical margin). […] Soft tissue sarcoma that is completely removed by surgery has a much more favourable prognosis than soft tissue sarcoma that is not completely removed or if surgery cant be done. […] People with soft tissue sarcoma who are under the age of 50 tend to have a better prognosis than those over the age of 50.
  • #39 Prognosis and survival for soft tissue sarcoma | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/soft-tissue-sarcoma/prognosis-and-survival
    If cancer cells are found in the margin (called a positive surgical margin), there is a poorer prognosis compared to when there are no cancer cells in the margin (called a negative surgical margin). […] Soft tissue sarcoma that is completely removed by surgery has a much more favourable prognosis than soft tissue sarcoma that is not completely removed or if surgery cant be done. […] People with soft tissue sarcoma who are under the age of 50 tend to have a better prognosis than those over the age of 50.
  • #40 Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report
    https://www.mdpi.com/2077-0383/12/17/5632
    There is a reported potential benefit of adjuvant radiotherapy in reducing recurrence after surgical resection, and radiotherapy is routinely used as an adjuvant to surgery. […] The tumor in this case exhibited an aggressive behavior, with enlarging local recurrence, metastatic spread, and dissemination within the rest of the central nervous system even while undergoing radiotherapy. […] The patient’s clinical course in this case mirrors that in the literature, with a poor overall prognosis, high local recurrence rate, and propensity for metastatic spread. […] The role of adjuvant chemotherapy in ES remains uncertain in the literature. Existing retrospective studies have suggested that chemotherapy does not have any survival benefit. […] Overall, the benefit of adjuvant chemotherapy in ES of the spine remains unclear.
  • #41 Soft Tissue Sarcoma Treatment (PDQ®) – NCI
    https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq
    Surgical resection and lymphadenectomy with or without postoperative radiation therapy may be indicated for patients with clinically positive lymph nodes.[1] […] Neoadjuvant chemotherapy with or without radiation therapy or radiation therapy alone may be considered in selected cases where a limb-sparing surgery is advisable and/or there is a high probability of surgical resection with positive margins.[2-7] […] Adjuvant chemotherapy may be considered but is not known to improve overall survival.[1,8-11] […] The combination of gemcitabine and docetaxel is used as second-line therapy in treating patients with soft tissue sarcoma. […] Although doxorubicin alone has traditionally been considered the standard when comparing new drugs or regimens in the context of phase III clinical trials, some sarcoma subtypes have shown higher sensitivity to specific agents. […] Epithelioid sarcoma Tazemetostat [27]
  • #42 Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report
    https://www.mdpi.com/2077-0383/12/17/5632
    There is a reported potential benefit of adjuvant radiotherapy in reducing recurrence after surgical resection, and radiotherapy is routinely used as an adjuvant to surgery. […] The tumor in this case exhibited an aggressive behavior, with enlarging local recurrence, metastatic spread, and dissemination within the rest of the central nervous system even while undergoing radiotherapy. […] The patient’s clinical course in this case mirrors that in the literature, with a poor overall prognosis, high local recurrence rate, and propensity for metastatic spread. […] The role of adjuvant chemotherapy in ES remains uncertain in the literature. Existing retrospective studies have suggested that chemotherapy does not have any survival benefit. […] Overall, the benefit of adjuvant chemotherapy in ES of the spine remains unclear.
  • #43 Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report
    https://www.mdpi.com/2077-0383/12/17/5632
    There is a reported potential benefit of adjuvant radiotherapy in reducing recurrence after surgical resection, and radiotherapy is routinely used as an adjuvant to surgery. […] The tumor in this case exhibited an aggressive behavior, with enlarging local recurrence, metastatic spread, and dissemination within the rest of the central nervous system even while undergoing radiotherapy. […] The patient’s clinical course in this case mirrors that in the literature, with a poor overall prognosis, high local recurrence rate, and propensity for metastatic spread. […] The role of adjuvant chemotherapy in ES remains uncertain in the literature. Existing retrospective studies have suggested that chemotherapy does not have any survival benefit. […] Overall, the benefit of adjuvant chemotherapy in ES of the spine remains unclear.
  • #44 Soft Tissue Sarcoma Treatment (PDQ®) – NCI
    https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq
    Surgical resection and lymphadenectomy with or without postoperative radiation therapy may be indicated for patients with clinically positive lymph nodes.[1] […] Neoadjuvant chemotherapy with or without radiation therapy or radiation therapy alone may be considered in selected cases where a limb-sparing surgery is advisable and/or there is a high probability of surgical resection with positive margins.[2-7] […] Adjuvant chemotherapy may be considered but is not known to improve overall survival.[1,8-11] […] The combination of gemcitabine and docetaxel is used as second-line therapy in treating patients with soft tissue sarcoma. […] Although doxorubicin alone has traditionally been considered the standard when comparing new drugs or regimens in the context of phase III clinical trials, some sarcoma subtypes have shown higher sensitivity to specific agents. […] Epithelioid sarcoma Tazemetostat [27]
  • #45 Pazopanib in rare histologies of metastatic soft tissue sarcoma – ecancer
    https://ecancer.org/en/journal/article/1281-pazopanib-in-rare-histologies-of-metastatic-soft-tissue-sarcoma
    Epithelioid sarcoma is refractory to conventional chemotherapy with a reported median survival of 52 weeks with palliative chemotherapy. […] In our cohort of 33 patients on pazopanib, we reported a longer median PFS of 10.2 months compared to the pazopanib for metastatic soft-tissue sarcoma (PALETTE) trial and other reported studies on the use of pazopanib. […] Our study observed an ORR of 27%, all of which were PRs. Our results are comparable with other published reports on pazopanib which is better than chemotherapy for most subtypes. […] We observed a median OS of 17.8 months in our study cohort. Most published studies have either not reported median OS or had a short follow-up, where the median OS was not reached. […] The positive result of our study suggests that the rare subtypes of STS, which are usually chemo-resistant, may be relatively more sensitive to pazopanib.
  • #46 Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report
    https://www.mdpi.com/2077-0383/12/17/5632
    There is a reported potential benefit of adjuvant radiotherapy in reducing recurrence after surgical resection, and radiotherapy is routinely used as an adjuvant to surgery. […] The tumor in this case exhibited an aggressive behavior, with enlarging local recurrence, metastatic spread, and dissemination within the rest of the central nervous system even while undergoing radiotherapy. […] The patient’s clinical course in this case mirrors that in the literature, with a poor overall prognosis, high local recurrence rate, and propensity for metastatic spread. […] The role of adjuvant chemotherapy in ES remains uncertain in the literature. Existing retrospective studies have suggested that chemotherapy does not have any survival benefit. […] Overall, the benefit of adjuvant chemotherapy in ES of the spine remains unclear.
  • #47 Recent advances in sarcoma therapy: new agents, strategies and predictive biomarkers | Journal of Hematology & Oncology | Full Text
    https://jhoonline.biomedcentral.com/articles/10.1186/s13045-024-01650-6
    Epithelioid sarcoma (ES) is an aggressive STS, often resistant to chemotherapy and radiotherapy, with limited treatment options. […] Tazmetostat, an EZH2 inhibitor, was approved for advanced ES based on a basket trial with 62 patients treated, with ORR of 14.5% (9/62), median PFS of 5.5 months and median OS of 19 months. […] Zhou et al. reported a phase 2 trial of SHR-2554, an oral selective EZH2 inhibitor, in advanced ES. Fourteen patients with advanced ES were treated, 3 obtained a PR, and AEs were mostly expected. The results appeared similar to that with Tazmetostat.
  • #48 Recent advances in sarcoma therapy: new agents, strategies and predictive biomarkers | Journal of Hematology & Oncology | Full Text
    https://jhoonline.biomedcentral.com/articles/10.1186/s13045-024-01650-6
    Epithelioid sarcoma (ES) is an aggressive STS, often resistant to chemotherapy and radiotherapy, with limited treatment options. […] Tazmetostat, an EZH2 inhibitor, was approved for advanced ES based on a basket trial with 62 patients treated, with ORR of 14.5% (9/62), median PFS of 5.5 months and median OS of 19 months. […] Zhou et al. reported a phase 2 trial of SHR-2554, an oral selective EZH2 inhibitor, in advanced ES. Fourteen patients with advanced ES were treated, 3 obtained a PR, and AEs were mostly expected. The results appeared similar to that with Tazmetostat.
  • #49 Recent advances in sarcoma therapy: new agents, strategies and predictive biomarkers | Journal of Hematology & Oncology | Full Text
    https://jhoonline.biomedcentral.com/articles/10.1186/s13045-024-01650-6
    Epithelioid sarcoma (ES) is an aggressive STS, often resistant to chemotherapy and radiotherapy, with limited treatment options. […] Tazmetostat, an EZH2 inhibitor, was approved for advanced ES based on a basket trial with 62 patients treated, with ORR of 14.5% (9/62), median PFS of 5.5 months and median OS of 19 months. […] Zhou et al. reported a phase 2 trial of SHR-2554, an oral selective EZH2 inhibitor, in advanced ES. Fourteen patients with advanced ES were treated, 3 obtained a PR, and AEs were mostly expected. The results appeared similar to that with Tazmetostat.
  • #50 Pazopanib in rare histologies of metastatic soft tissue sarcoma – ecancer
    https://ecancer.org/en/journal/article/1281-pazopanib-in-rare-histologies-of-metastatic-soft-tissue-sarcoma
    Epithelioid sarcoma is refractory to conventional chemotherapy with a reported median survival of 52 weeks with palliative chemotherapy. […] In our cohort of 33 patients on pazopanib, we reported a longer median PFS of 10.2 months compared to the pazopanib for metastatic soft-tissue sarcoma (PALETTE) trial and other reported studies on the use of pazopanib. […] Our study observed an ORR of 27%, all of which were PRs. Our results are comparable with other published reports on pazopanib which is better than chemotherapy for most subtypes. […] We observed a median OS of 17.8 months in our study cohort. Most published studies have either not reported median OS or had a short follow-up, where the median OS was not reached. […] The positive result of our study suggests that the rare subtypes of STS, which are usually chemo-resistant, may be relatively more sensitive to pazopanib.
  • #51 Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report
    https://www.mdpi.com/2077-0383/12/17/5632
    Epithelioid sarcoma is a rare malignant mesenchymal tumor that represents less than 1% of soft-tissue sarcomas. Despite its slow growth, the overall prognosis is poor with a high rate of local recurrence, lymph-node spread, and hematogenous metastasis. […] The disease has a poor prognosis, with high propensity for local recurrence, as well as lymphatic and hematogenous metastatic spread. […] Recurrence is often noted to occur, with a recurrence rate varying from 34% and 77% depending on the adequacy of initial excision. […] Metastasis occurs in 40% of patients, predominantly involving the lungs, regional nodes, scalp, bone, and brain. […] Poor prognostic factors for ES in the literature include a proximal or axial tumor location, increased size and depth, hemorrhage, mitotic figures, necrosis, presence of vascular invasion, and the presence of distant metastases at the time of diagnosis.
  • #52 Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report
    https://www.mdpi.com/2077-0383/12/17/5632
    Epithelioid sarcoma is a rare malignant mesenchymal tumor that represents less than 1% of soft-tissue sarcomas. Despite its slow growth, the overall prognosis is poor with a high rate of local recurrence, lymph-node spread, and hematogenous metastasis. […] The disease has a poor prognosis, with high propensity for local recurrence, as well as lymphatic and hematogenous metastatic spread. […] Recurrence is often noted to occur, with a recurrence rate varying from 34% and 77% depending on the adequacy of initial excision. […] Metastasis occurs in 40% of patients, predominantly involving the lungs, regional nodes, scalp, bone, and brain. […] Poor prognostic factors for ES in the literature include a proximal or axial tumor location, increased size and depth, hemorrhage, mitotic figures, necrosis, presence of vascular invasion, and the presence of distant metastases at the time of diagnosis.
  • #53 The diagnosis and treatment of the epithelioid sarcomas involving the peripheral nerves | Scientific Reports
    https://www.nature.com/articles/s41598-024-82357-z
    Epithelioid sarcomas are rare soft tissue tumors and have possibility to involve the peripheral nerve and present as sensory and motor disorders. […] Early diagnosis and treatment are the keys to better prognosis. […] The symptoms presented were similar to those of the nerve compression diseases, which usually led to misdiagnosis and mistreatment. Early diagnosis and early treatment are the keys to better prognosis. […] The overall 1-year, 3-year and 5-year survival were 70.7, 56.1 and 50.4%, respectively. The overall recurrence and metastasis rates were 63.4% and 40.3%. Age more than 56 years old, higher tumor grade, tumor size over 5 cm and the presence of metastasis are related to poor prognosis. […] We should not ignore the lymph node metastasis pre- and postoperatively. The tumor should be resected together with the peripheral nerve involved. A negative surgical margin according to the frozen pathology is essential during the operation. […] Postoperative chemotherapy and radiotherapy are still controversial in the literature. The patients need close follow-up to discovered the local recurrence and/or lymph node metastasis as early as possible.
  • #54 The diagnosis and treatment of the epithelioid sarcomas involving the peripheral nerves | Scientific Reports
    https://www.nature.com/articles/s41598-024-82357-z
    Epithelioid sarcomas are rare soft tissue tumors and have possibility to involve the peripheral nerve and present as sensory and motor disorders. […] Early diagnosis and treatment are the keys to better prognosis. […] The symptoms presented were similar to those of the nerve compression diseases, which usually led to misdiagnosis and mistreatment. Early diagnosis and early treatment are the keys to better prognosis. […] The overall 1-year, 3-year and 5-year survival were 70.7, 56.1 and 50.4%, respectively. The overall recurrence and metastasis rates were 63.4% and 40.3%. Age more than 56 years old, higher tumor grade, tumor size over 5 cm and the presence of metastasis are related to poor prognosis. […] We should not ignore the lymph node metastasis pre- and postoperatively. The tumor should be resected together with the peripheral nerve involved. A negative surgical margin according to the frozen pathology is essential during the operation. […] Postoperative chemotherapy and radiotherapy are still controversial in the literature. The patients need close follow-up to discovered the local recurrence and/or lymph node metastasis as early as possible.
  • #55 Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database
    https://pmc.ncbi.nlm.nih.gov/articles/PMC2758965/
    Current stratification of prognosis in patients with epithelioid sarcoma (ES) is based largely on data reported by individual centers with a limited number of patients. […] On multivariate analysis, only age younger than 16 years, local stage of disease, or negative nodes and surgical resection of the tumor predicted better disease-specific survival. […] The SEER database shows only age younger than 16 years, negative nodes, or local stage of disease and operability of primary disease independently predict survival in patients with ES. […] The disease-specific survival was 68% at 5 years and 61% at 10 years, and started to plateau at approximately 100 months, which suggests deaths occurring thereafter can be attributed to factors other than ES. […] Patients diagnosed with local disease had a better 5-year survival of 75% as compared with patients with regional disease who had a 5-year survival of 49%.
  • #56 Epithelioid Sarcoma: Symptoms, Treatment & Prognosis
    https://my.clevelandclinic.org/health/diseases/24331-epithelioid-sarcoma
    Epithelioid sarcoma is treatable, especially when detected early. […] The only way to cure ES is to completely remove the tumor. But complete removal isn’t possible in all cases. […] Because ES is slow growing, many people with the disease can still lead long, fulfilling lives. […] The five-year survival rate for epithelioid sarcoma ranges from 25% to 92%, depending on which study you read.

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