Sarcoma nabłonkowate
Epidemiologia
Sarcoma nabłonkowate (ES) to rzadki, agresywny mięsak tkanek miękkich o pochodzeniu mezenchymalnym, charakteryzujący się utratą ekspresji białka SMARCB1/INI1. Występuje głównie u młodych dorosłych (mediana wieku 27 lat), z częstością zachorowań wynoszącą 0,02-0,05/100 000 w UE i USA. Wyróżnia się dwa podtypy: klasyczny (dystalny) i proksymalny, z przewagą zachorowań u mężczyzn (stosunek 2:1). ES cechuje się wysokim ryzykiem nawrotów miejscowych (40-60%) i przerzutów regionalnych do węzłów chłonnych (do 30%), a także odległych (płuca, kości, mózg) u 20-50% pacjentów. 5-letnie przeżycie całkowite waha się od 25% do 70%, zależnie od stopnia zaawansowania choroby i obecności przerzutów. Czynniki pogarszające rokowanie to m.in. wielkość guza >5 cm, lokalizacja proksymalna, obecność nacieków naczyniowych, martwica ≥30%, dodatnie marginesy chirurgiczne oraz wysoka aktywność mitotyczna.
Epidemiologia sarcoma nabłonkowatego
Sarcoma nabłonkowate (epithelioid sarcoma, ES) jest rzadkim nowotworem tkanek miękkich pochodzenia mezenchymalnego, charakteryzującym się utratą ekspresji białka SMARCB1/INI1 lub innych białek kompleksu SWI/SNF. Stanowi mniej niż 1% wszystkich mięsaków tkanek miękkich u dorosłych oraz około 4-8% mięsaków tkanek miękkich innych niż rhabdomyosarcoma u dzieci.123
Częstotliwość występowania
Współczynnik zachorowalności na sarcoma nabłonkowate wynosi 0,03/100 000 w Unii Europejskiej i 0,05/100 000 w USA według rejestrów RARECAREnet i SEER18. Standaryzowany względem wieku współczynnik zachorowalności wynosi odpowiednio 0,02/100 000 i 0,05/100 000.1 Według danych z bazy SEER, ogólna częstość występowania ES w 2005 roku wynosiła 0,041 na 100 000 mieszkańców, a zgłaszana zachorowalność wzrasta od 1973 roku, z roczną zmianą procentową wynoszącą 5,217%.2
Według szacunków DelveInsight, w Stanach Zjednoczonych w 2021 roku odnotowano 68 przypadków ES, co stanowiło 37% wszystkich przypadków w tzw. 7MM (siedmiu głównych rynkach). Wśród krajów UE-5, najwięcej przypadków ES w 2021 roku zgłoszono w Niemczech (24 przypadki), następnie we Włoszech (21 przypadków) i we Francji (20 przypadków). Najmniej przypadków odnotowano w Hiszpanii (13 przypadków).3
Charakterystyka demograficzna
Sarcoma nabłonkowate najczęściej występuje u młodych dorosłych, ze szczytem zachorowalności przypadającym na 35 rok życia.1 Choroba zazwyczaj rozwija się u osób młodych i w średnim wieku (głównie w grupie wiekowej 20-40 lat).1 Zakres wieku pacjentów z ES jest szeroki (od 4 do 90 lat), z medianą wieku wynoszącą 27 lat.45
Wyróżnia się dwa główne podtypy sarcoma nabłonkowatego:16
- Podtyp klasyczny (dystalny) – występuje głównie u nastolatków i młodych dorosłych, szczególnie w obrębie kończyn
- Podtyp proksymalny – występuje częściej u osób młodych i w średnim wieku, z tendencją do lokalizacji w bliższych częściach ciała
Mężczyźni chorują na sarcoma nabłonkowate częściej niż kobiety, z proporcją 2:1.1 W przypadku podtypu klasycznego stosunek mężczyzn do kobiet wynosi 1,9:1, a w przypadku podtypu proksymalnego 1,6:1.2 Według niektórych źródeł, podtyp klasyczny jest prawie dwa razy częstszy niż podtyp proksymalny.61
Przebieg kliniczny i rokowanie
Sarcoma nabłonkowate charakteryzuje się agresywnym przebiegiem z wysokim odsetkiem nawrotów miejscowych i przerzutów regionalnych do węzłów chłonnych.1 W momencie rozpoznania choroba zwykle objawia się jako niebolesny, wolno rosnący, twardy guzek zlokalizowany głęboko w tkankach miękkich, o połyskliwym i szaro-brązowym wyglądzie, charakteryzujący się powierzchownym krwawieniem, martwicą i owrzodzeniami.1
ES często daje przerzuty do węzłów chłonnych (nawet do 30% przypadków).1 W porównaniu do innych typów mięsaków tkanek miękkich, rozprzestrzenianie się do regionalnych węzłów chłonnych występuje częściej (około 20%).7 Pacjenci cierpią z powodu przerzutów in-transit i rozprzestrzeniania się limfatycznego. Odległe przerzuty najczęściej rozwijają się w płucach, kościach i mózgu.1
Przerzuty odległe stwierdza się w przebiegu choroby u 20% do 50% pacjentów. Około 20% pacjentów ma już przerzuty odległe w momencie pierwotnej diagnozy.18 Według niektórych źródeł, z powodu opóźnień w rozpoznaniu sarcoma nabłonkowatego, u około 1/3 pacjentów w momencie pierwotnego zgłoszenia wykrywa się przerzuty do węzłów chłonnych, a u 1/4 pacjentów przerzuty do płuc.9
5-letnie ryzyko nawrotu może przekraczać 70%.1 Opisywane wskaźniki miejscowych nawrotów wahają się od 40% do 60%, z medianą czasu do nawrotu wynoszącą 1-2 lata. Jednakże nawroty odnotowywano nawet 20 lat po pierwotnej operacji.45
Wskaźniki przeżycia w sarcoma nabłonkowatym
Przeżycie pacjentów z sarcoma nabłonkowatym jest niezadowalające. W zależności od badania, 5-letnie wskaźniki przeżycia całkowitego wahają się od 25% do 70%.110 Ogólne wskaźniki przeżycia 5-letniego wynoszą 60,4%, a 10-letniego 50,2%. Ogólny wskaźnik nawrotów wynosi 63,4%, a wskaźnik przerzutów 40,3%.11
Wskaźniki przeżycia różnią się w zależności od stopnia zaawansowania choroby:9
- 70% 5-letnie przeżycie dla pacjentów z chorobą miejscową
- 50% 5-letnie przeżycie dla pacjentów z zajęciem regionalnych węzłów chłonnych
- W przypadku pacjentów z przerzutami do płuc, jednoroczne przeżycie wynosi 50%, a pięcioletnie przeżycie jest bliskie zeru
Czynniki prognostyczne
Liczne czynniki wpływają na rokowanie w sarcoma nabłonkowatym:6129
- Choroba przerzutowa – obecność przerzutów odległych i zajęcie węzłów chłonnych znacząco pogarszają rokowanie
- Lokalizacja guza – guzy głęboko położone lub typu proksymalnego wiążą się z gorszym rokowaniem
- Wielkość guza – większe guzy (>5 cm) są związane z gorszym rokowaniem
- Wiek pacjenta – starszy wiek w momencie diagnozy wiąże się z gorszym rokowaniem
- Płeć – mężczyźni mają gorsze rokowanie niż kobiety
- Nacieki naczyniowe – obecność nacieków naczyniowych pogarsza rokowanie
- Martwica – wysoki odsetek martwicy (≥30%) wiąże się z gorszym rokowaniem
- Margines chirurgiczny – dodatnie marginesy chirurgiczne (resekcje R1 i R2) są związane z wyższym ryzykiem nawrotu
- Wysoka aktywność mitotyczna komórek nowotworowych negatywnie wpływa na rokowanie
Nadzór i monitorowanie pacjentów z sarcoma nabłonkowatym
Ze względu na wysokie prawdopodobieństwo późnych przerzutów i nawrotów, długoterminowe monitorowanie jest niezbędne w zarządzaniu chorobą.4 Zaleca się ścisły nadzór z obrazowaniem przekrojowym miejsca pierwotnego guza i płuc, zazwyczaj z badaniami obrazowymi wykonywanymi co 6 miesięcy do 1 roku.45
Ponieważ nawroty miejscowe zwykle pojawiają się w ciągu 1-2 lat od leczenia, a pacjenci często rozwijają później przerzuty odległe, ścisła, długoterminowa obserwacja jest konieczna.13 Należy jednak pamiętać, że nawroty mogą wystąpić nawet 20 lat po pierwotnym leczeniu, co podkreśla konieczność długotrwałego nadzoru.5
Metody obrazowania w monitorowaniu
W monitorowaniu pacjentów z sarcoma nabłonkowatym stosuje się różnorodne techniki obrazowania:57
- Tomografia komputerowa (TK) klatki piersiowej – podstawowe badanie w ocenie przerzutów do płuc, które są najczęstszą lokalizacją przerzutów odległych
- Obrazowanie przekrojowe miejsca pierwotnego guza – do oceny nawrotów miejscowych
- Pozytonowa tomografia emisyjna (PET-FDG) – szczególnie przydatna w przypadku podtypów mięsaków z predyspozycją do przerzutów do węzłów chłonnych, jak sarcoma nabłonkowate
- Dodatkowe badania TK innych lokalizacji – w zależności od charakterystyki guza pierwotnego i objawów klinicznych
System stadiowania w monitorowaniu
Stadiowanie mięsaków tkanek miękkich, w tym sarcoma nabłonkowatego, opiera się na systemie TNM Amerykańskiego Wspólnego Komitetu ds. Raka (AJCC), gdzie T oznacza wielkość guza pierwotnego, N oznacza zajęcie regionalnych węzłów chłonnych, a M oznacza obecność choroby przerzutowej.1014
System AJCC wyznacza stopień zaawansowania według następujących kryteriów: wielkość guza, status węzłów chłonnych, przerzuty i stopień histologiczny (TNMG).14 Stopień histologiczny mięsaka jest ważnym czynnikiem w stadiowaniu wszystkich mięsaków tkanek miękkich, a mięsaki tkanek miękkich miejscowo zaawansowane o wysokim stopniu złośliwości mają zwiększony potencjał do nawrotu miejscowego i przerzutów.14
Wyzwania w monitorowaniu i nadzorze
Ze względu na rzadkość sarcoma nabłonkowatego, monitorowanie pacjentów napotyka liczne wyzwania:151617
- Brak konsensusowych wytycznych dotyczących leczenia systemowego, stadiowania i obrazowania pacjentów z ES
- Przedłużony proces diagnostyczny w porównaniu do innych nowotworów
- Większe opóźnienie diagnostyczne u pacjentów z ograniczeniami w ubezpieczeniu zdrowotnym, starszych populacji i z niższym statusem społeczno-ekonomicznym
- Konieczność podróżowania do ośrodków z dedykowanym multidyscyplinarnym programem mięsakowym, posiadającym doświadczenie w diagnozowaniu i leczeniu mięsaków
- Bariery geograficzne i transportowe mające kluczowe znaczenie dla poprawy wyników leczenia pacjentów z ES
- Szczególne potrzeby młodych dorosłych pacjentów (AYA) związane z ich trajektoriami społecznymi i rozwojowymi
- Poczucie izolacji wśród pacjentów z ES, spotęgowane rzadkością choroby
Zwiększenie świadomości i zrozumienia sarcoma nabłonkowatego jest niezbędnym pierwszym krokiem do poprawy procesu diagnostycznego poprzez: (1) skrócenie czasu do prawidłowej diagnozy, (2) zmniejszenie barier w dostępie do terminowego leczenia i doświadczonych ośrodków mięsakowych, oraz (3) zwiększenie świadomości wśród lekarzy podstawowej opieki zdrowotnej i innych świadczeniodawców, którzy mogą być pierwszym punktem kontaktu dla tych pacjentów.17
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Materiały źródłowe
- #1 Epithelioid Sarcoma – SFAhttps://curesarcoma.org/sarcoma-subtypes/epithelioid-sarcoma/
Epidemiology […] ES represents < 1% of all adult soft tissue sarcomas and between 4% and 8% of childhood non-rhabdomyosarcomatous soft tissue sarcomas. The classic subtype is reported nearly twice as often as the proximal-type subtype. The M:F ratio is 2:1 for the classic subtype and 1.6:1 for the proximal-type subtype. Both tumors affect patients over a wide age range. The classic subtype presents mainly in adolescents and young adults, whereas the proximal-type subtype tends to affect a somewhat older population â young to middle-aged adults.
- #1 Epithelioid SarcomaâFrom Genetics to Clinical Practicehttps://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/
Epithelioid sarcoma is a mesenchymal soft tissue sarcoma often arising in the extremities, usually in young adults with a pick of incidence at 35 years of age. Epithelioid sarcoma (ES) is characterized by the loss of SMARCB1/INI1 (integrase interactor 1) or other proteins of the SWI/SNF complex. ES is known for aggressive behavior, including a high recurrence rate and regional lymph node metastases. The ES incidence rate is 0.03/100,000 in the European Union and 0.05/10,000 in the USA as per RARECAREnet and the SEER18 cancer registries. Age-adjusted rate in EU and USA is 0.02/100,000 and 0.05/100,000, respectively. ES usually develops in young to middle-aged adults (20-40 years of age group). Males suffer from ES more often than females, with a ratio of 2:1. At presentation, the disease typically manifests as a painless, slow-growing, firm nodule deep in soft tissues with a glistening and gray-tan appearance characterized by superficial bleeding, necrosis, and ulcerations. ES often metastasize to lymph nodes (up to 30%). Patients suffer from in-transit metastases and lymphatic spread. Distant metastases most often develop in the lungs, bones, and brain. ES metastatic spread is reported in the course of the disease in 20% to 50% of patients. Around 20% of patients present with distant metastases already at the primary diagnosis. The treatment of choice of ES is a radical excision with microscopically radical margins and perioperative radiotherapy after careful assessment in a multidisciplinary team. As compared to other types of soft tissue sarcomas, the spread to regional lymph nodes can occur more frequently (20%). The 5-year risk of recurrence can even exceed 70%. Positive surgical margins (R1 and R2 resections) were associated with a higher risk of recurrence. ES is generally considered radioresistant, even in comparison to the rest of soft tissue sarcomas. However, data related to definitive or perioperative RT in ES are scarce. The largest analysis found in the literature described 24 patients with nonmetastatic ES treated with perioperative RT and surgery. Among them, only three patients received neoadjuvant RT with a median total dose of 46.4 Gy. The rest were irradiated postoperatively, with a median total dose of 64.5 Gy. Disappointingly, local failure occurred in 7 (29%) patients, which translated into a 10-year local control rate of 63%. In comparison to other soft tissue sarcomas with a local control rate of over 80%, that result seems to be unsatisfactory. 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%.
- #2 Pathology Outlines – Epithelioid sarcomahttps://www.pathologyoutlines.com/topic/softtissueepithelioidsarcoma.html
Epidemiology […] Rare, < 1% of all adult soft tissue sarcomas, 4 - 8% of pediatric non rhabdomyoblastic sarcomas. [...] Classic type epithelioid sarcoma is more common than the proximal type epithelioid sarcoma. [...] Classic type: Most common in adolescents and young adults, between ages 10 and 40 years; M:F = 1.9:1 (Adv Anat Pathol 2016;23:41). [...] Proximal type: Middle aged to older adults, > 80% presenting between ages of 20 and 65 years; M:F = 1.6:1.
- #2 Prognostic Factors for Survival in Patients with Epithelioid Sarcoma: 441 Cases from the SEER Database | springermedicine.comhttps://www.springermedicine.com/prognostic-factors-for-survival-in-patients-with-epithelioid-sar/21973710
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. […] We sought to identify the important prognostic parameters using the Surveillance, Epidemiology, and End Results (SEER) database. […] The overall incidence of ES during 2005 was 0.041 per 100,000. […] The reported incidence has increased since 1973, with an annual percentage change of 5.217%. […] 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.
- #3 Epithelioid SarcomaâFrom Genetics to Clinical Practicehttps://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/
Epithelioid sarcoma is a mesenchymal soft tissue sarcoma often arising in the extremities, usually in young adults with a pick of incidence at 35 years of age. Epithelioid sarcoma (ES) is characterized by the loss of SMARCB1/INI1 (integrase interactor 1) or other proteins of the SWI/SNF complex. ES is known for aggressive behavior, including a high recurrence rate and regional lymph node metastases. The ES incidence rate is 0.03/100,000 in the European Union and 0.05/10,000 in the USA as per RARECAREnet and the SEER18 cancer registries. Age-adjusted rate in EU and USA is 0.02/100,000 and 0.05/100,000, respectively. ES usually develops in young to middle-aged adults (20-40 years of age group). Males suffer from ES more often than females, with a ratio of 2:1. At presentation, the disease typically manifests as a painless, slow-growing, firm nodule deep in soft tissues with a glistening and gray-tan appearance characterized by superficial bleeding, necrosis, and ulcerations. ES often metastasize to lymph nodes (up to 30%). Patients suffer from in-transit metastases and lymphatic spread. Distant metastases most often develop in the lungs, bones, and brain. ES metastatic spread is reported in the course of the disease in 20% to 50% of patients. Around 20% of patients present with distant metastases already at the primary diagnosis. The treatment of choice of ES is a radical excision with microscopically radical margins and perioperative radiotherapy after careful assessment in a multidisciplinary team. As compared to other types of soft tissue sarcomas, the spread to regional lymph nodes can occur more frequently (20%). The 5-year risk of recurrence can even exceed 70%. Positive surgical margins (R1 and R2 resections) were associated with a higher risk of recurrence. ES is generally considered radioresistant, even in comparison to the rest of soft tissue sarcomas. However, data related to definitive or perioperative RT in ES are scarce. The largest analysis found in the literature described 24 patients with nonmetastatic ES treated with perioperative RT and surgery. Among them, only three patients received neoadjuvant RT with a median total dose of 46.4 Gy. The rest were irradiated postoperatively, with a median total dose of 64.5 Gy. Disappointingly, local failure occurred in 7 (29%) patients, which translated into a 10-year local control rate of 63%. In comparison to other soft tissue sarcomas with a local control rate of over 80%, that result seems to be unsatisfactory. 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%.
- #3 Epithelioid Sarcoma Treatment Market 2032: EMA, PDMA, FDAhttps://www.openpr.com/news/3994587/epithelioid-sarcoma-treatment-market-2032-ema-pdma-fda
Epithelioid Sarcoma Companies are Epizyme, Eli Lilly and Company, Pfizer, Merck Sharp Dohme, GlaxoSmithKline and Among Others. DelveInsight’s „Epithelioid Sarcoma Market Insights, Epidemiology, and Market Forecast-2032 report offers an in-depth understanding of the Epithelioid Sarcoma, historical and forecasted epidemiology as well as the Epithelioid Sarcoma market trends in the United States, EU4 (Germany, Spain, Italy, France, and United Kingdom) and Japan. […] As per the estimation of DelveInsight, there were 68 cases of ES in 2021, in the US, which was 37% of overall cases of 7MM. […] According to DelveInsight’s estimation, 68 occurrences of ES, or 37% of all cases of 7MM, were reported in the US in 2021. […] Germany reported the most ES cases among the EU-5 in 2021 (24 cases), followed by Italy (21 instances) and France (20). Spain has the fewest cases (13 cases).
- #4 Epithelioid Sarcoma – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK532911/
Epithelioid sarcomas are rare, accounting for less than 1% of soft tissue sarcomas. They predominantly affect men, with a male-to-female ratio of up to 2:1. Most reported tumors occur in young males aged 10 to 45. The age range spans from 4 to 90, with a median age of 27. […] Epithelioid sarcoma is locally invasive and frequently metastasizes to regional lymph nodes and distant sites. Diagnosis is determined by tissue biopsy, while imaging studies are essential for assessing the extent of the primary tumor and identifying metastatic disease. […] Due to the high likelihood of late metastases, long-term monitoring is essential for managing the disease. […] Local recurrence rates of 40% to 60% have been reported, with a median time to recurrence of 1 to 2 years. However, recurrences have been reported as late as 20 years after the initial operation. As a result, close surveillance with cross-sectional imaging of the primary site and lungs is critical, typically with imaging studies every 6 months to 1 year.
- #5 Epithelioid Sarcoma | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/449
Epithelioid sarcomas are rare, accounting for less than 1% of soft tissue sarcomas. They predominantly affect men, with a male-to-female ratio of up to 2:1. Most reported tumors occur in young males aged 10 to 45. The age range spans from 4 to 90, with a median age of 27. […] Epithelioid sarcoma is associated with a high rate of distant metastases, with recent studies suggesting a distant metastasis rate of up to 50%. The most common sites of metastases are the lungs, lymph nodes, and skin. As a result, staging imaging with a chest computed tomography (CT) scan is typically performed. For more extensive staging, fluorodeoxyglucose-positron emission tomography (FDG-PET) scans or additional CT scans may be necessary, depending on the characteristics of the primary tumor. […] Local recurrence rates of 40% to 60% have been reported, with a median time to recurrence of 1 to 2 years. However, recurrences have been reported as late as 20 years after the initial operation. As a result, close surveillance with cross-sectional imaging of the primary site and lungs is critical, typically with imaging studies every 6 months to 1 year.
- #6 Epithelioid sarcoma | Radiology Reference Article | Radiopaedia.orghttps://radiopaedia.org/articles/epithelioid-sarcoma?embed_domain=hackmd.io%252525252F%2525252540yipuafecsl2jsu8smr5njq%252525252Fbnjhjgjghjghjghradiopaedia-icon-144.pngfavicon.icofavicon.ico&lang=us
Epithelioid sarcomas are rare and make up for 1% of soft tissue sarcomas. They are found in children and adults over a wide age range with the classic subtype featuring a peak incidence in adolescence and young adults and the proximal subtype showing its peak later. There is a male predilection. The classic subtype is apparently almost twice as common as the proximal subtype. […] Overall survival rates have been reported to be in the range of 45-70% and 45-66% for 5 and 10 years respectively. Metastatic disease, nodal involvement, deep-seated tumors or proximal type, high mitotic activity, greater tumor size as well as older age of the patient are associated with a worse prognosis.
- #7 Soft Tissue Sarcoma Treatment (PDQ®) – NCIhttps://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq
Estimated new cases and deaths from soft tissue sarcoma in the United States in 2025: New cases: 13,520. Deaths: 5,410. […] The reported international incidence of soft tissue sarcoma ranges from 1.8 to 5 cases per 100,000 individuals per year. The rate of new cases of soft tissue cancer in the United States was 3.4 per 100,000 people per year based on age-adjusted cases from 2017 to 2021. The death rate was 1.3 per 100,000 people per year based on age-adjusted deaths from 2018 to 2022. […] PET imaging is particularly useful for sarcoma subtypes with a propensity for lymph node metastases (e.g., synovial sarcoma, clear cell sarcoma, angiosarcoma, rhabdomyosarcoma, and epithelioid sarcoma). […] 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.
- #8 Treatment for Rare Type of Soft Tissue Sarcoma | The AACRhttps://www.aacr.org/patients-caregivers/progress-against-cancer/treatment-for-rare-type-of-soft-tissue-sarcoma/
Epithelioid sarcoma is a rare form of soft tissue sarcoma, accounting for less than 1 percent of the approximately 12,700 people in the United States diagnosed with soft tissue sarcomas each year. […] According to information in the FDAs approval notice, about 50 percent of patients have metastatic disease at the time they are diagnosed with epithelioid sarcoma and this is considered a life-threatening diagnosis.
- #9 EPITHELIOID SARCOMAhttps://www.seyitaligumustas.com/en/epithelioid-sarcoma
Epithelioid sarcoma is a very rare (less than 1% of all soft tissue sarcomas) malignant soft tissue tumor. Epithelioid sarcoma most commonly affects young adults (20-40 years old) and is slightly more common in men. […] Epithelioid sarcoma has an aggressive course, which means there is a high risk of recurrence after surgery and a high risk of spreading to other parts of the body (often lymph nodes and lungs). The average risk of recurrence after surgery is reported to be 50% (30-70) and the risk of metastasis is 40%. […] Due to the delay in diagnosis of epithelioid sarcoma, lymph node metastases are detected in approximately 1/3 of patients and lung metastases in 1/4 of patients at the time of initial admission. […] The average 5-year survival of patients with epithelioid sarcoma is 70% for patients with local disease and 50% for patients with regional lymph node involvement. In patients with pulmonary metastases, the one-year survival rate is 50% and the five-year survival rate is unfortunately close to zero. […] The prognosis is worse in the presence of tumor diameter 5cm, regional lymphadenopathy, vascular invasion, necrosis rate 30%, local symptoms, positive surgical margins and metastases.
- #10 An Overview of Epithelioid Sarcomahttps://www.pharmacytimes.com/view/an-overview-of-epithelioid-sarcoma
Epithelioid sarcoma (ES) is a rare, slow-growing subtype of STS that accounts for approximately 1% of all STS cases annually. […] It is estimated that 13,040 individuals received a diagnosis of STS in the United States in 2018 with a corresponding 5150 deaths. […] The 5-year overall survival for ES varies from 25% to 78%, dependent upon staging at the time of initial diagnosis. […] Staging of STS is based upon the American Joint Committee on Cancer TNM staging system, with T representing size of the primary tumor, N representing regional lymph node involvement, and M representing distant metastatic disease. […] Collectively, the TNM system allows an oncologist to categorize an STS patient as stage I, II, III, or IV. […] Tazemetostat was approved by the FDA on January 23, 2020, for the treatment of adult and pediatric patients 16 years and older with metastatic or locally advanced ES that is not eligible for complete resection. […] Prior to this pivotal approval, there were no medications with specific FDA-approved indications for use in the treatment of ES.
- #11https://medicaljournalssweden.se/actaoncologica/article/view/24055
Epithelioid sarcoma (ES) is an aggressive malignancy scarcely reported on due to its rarity. […] Data were acquired from both the Survival, Epidemiology, and End Results database and literature. […] Age, anatomical site, grade, TNM staging, treatment modality and year of diagnosis were demonstrated to be independent predictors of survival. Overall 5- and 10-year survival were 60.4% and 50.2%, respectively. Overall recurrence and metastasis rates were 63.4% and 40.3%. […] A worsening survival over the years warrants further investigation into this sarcoma.
- #12 Epithelioid sarcoma – Wikipediahttps://en.wikipedia.org/wiki/Epithelioid_sarcoma
Epithelioid sarcoma is a rare soft tissue sarcoma arising from mesenchymal tissue and characterized by epithelioid-like features. It accounts for less than 1% of all soft tissue sarcomas. […] 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.
- #13 Epithelioid Sarcoma: A Review and Update | JCAD – The Journal of Clinical and Aesthetic Dermatologyhttps://jcadonline.com/epithelioid-sarcoma-a-review-and-update/
Local recurrence often occurs within 1 to 2 years of treatment, and these patients often proceed to develop distant metastases. […] Anatomic location of ES appears to play a role in prognostication. The overall survival and metastases-free survival are worse in lesions proximal to the elbow or knee. […] Radical tumor excision is the primary treatment for patients with ES. […] Routine sentinel lymph node biopsy (SLNB) does not appear to be supported by data, though it is sometimes advocated due to the high rate of regional recurrence. […] Close, long-term follow up is necessary because recurrence and metastases may occur long after definitive treatment.
- #14 Soft Tissue Sarcoma Treatment (PDQ®) – NCIhttps://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq
The AJCC staging system has designated stage by the following criteria: Tumor size, nodal status, metastasis, histological grade (TNMG). […] The histological grade of the sarcoma is an important factor in staging all soft tissue sarcomas. […] High-grade localized soft tissue sarcomas have an increased potential for local recurrence and metastasis. […] Surgical resection and lymphadenectomy with or without postoperative radiation therapy may be indicated for patients with clinically positive lymph nodes. […] Epithelioid sarcomas. […] 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. […] Tazemetostat in advanced epithelioid sarcoma with loss of INI1/SMARCB1: an international, open-label, phase 2 basket study.
- #15 Recommendations from the Epithelioid Sarcoma Collaborative: A White Paper – Journal of Oncology Navigation & Survivorshiphttps://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/3914-recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper
Epithelioid sarcoma (ES) is a rare soft tissue sarcoma (STS) first described in the 1970s by Enzinger. STSs comprise approximately 1% of adult cancers and 7% to 15% of pediatric cancers annually, and ES accounts for 1% of STS diagnoses. […] The rarity of ES leads to multiple challenges for patients diagnosed with ES beginning with but not limited to the diagnosis journey, confounded by the subdivision of patients into 2 subtypes distal (classic) ES and proximal-type ES, and by the presentation. […] The suite of nonspecific symptoms of ES necessitates pathologic diagnosis, which is done by examining a piece of tissue from the lump or growth. […] Improved ES patient staging and imaging would improve patients initial diagnoses and their subsequent treatment assessment. […] Currently, there are no consensus guidelines for the systemic treatment, staging, and imaging of ES patients.
- #16 Recommendations from the Epithelioid Sarcoma Collaborative: A White Paper – Journal of Oncology Navigation & Survivorshiphttps://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/3914-recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper
The diagnostic journey overall is recognized to be prolonged among patients diagnosed with a sarcoma compared with other cancers. […] Greater diagnostic delay has also been recognized to be associated with patients with limitations in healthcare coverage, older populations, and lower socioeconomic profiles (ie, Medicare and Medicaid). […] The rarity of ES necessitates the involvement of a specialist in sarcoma, resulting in the need to travel to a center with a dedicated multidisciplinary sarcoma program that has expertise and experience in diagnosis and treatment of sarcoma. […] A recent analysis of the SEER database revealed a disparity in disease-specific survival among patients with sarcoma who had Medicaid insurance and those with non-Medicaid insurance, citing diagnosis and treatment delays, distance traveled to treatments and transportation barriers, out-of-pocket burdens, and education and literacy status as potential targets.
- #17 Recommendations from the Epithelioid Sarcoma Collaborative: A White Paper – Journal of Oncology Navigation & Survivorshiphttps://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/3914-recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper
Overcoming the system-level barriers of geographical access and transportation burden are key factors in improving outcomes for patients with ES. […] AYA patients diagnosed with cancer are recognized as having needs specific to their social and developmental trajectories and encountering barriers to care, both of which contribute to the diagnosis journey. […] The rarity of ES further exacerbates feelings of isolation among ES patients and leaves AYA programs and services lacking ES-specific care. […] In conclusion, increasing awareness and understanding of ES is a necessary first step to improve the diagnosis journey by (1) decreasing the time to correct diagnosis; (2) reducing barriers to timely treatment and access to expert, experienced sarcoma centers; and (3) raising awareness among GPs and other care providers who may be the first point of contact for these patients.