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Epithelioid sarcoma is a rare soft tissue cancer that has lost a gene brake called SMARCB1, leaving it dependent on the enzyme EZH2. Surgery cures localised tumours. The EZH2 inhibitor tazemetostat was approved in 2020 and withdrawn worldwide in March 2026 after secondary blood cancers in a lymphoma trial; the dependency it proved is still a target in development, and chemotherapy remains in use.
Epithelioid sarcoma is defined by loss of SMARCB1 (INI1), a core subunit of the SWI/SNF chromatin-remodelling complex, in over 90 percent of cases. SWI/SNF loss makes cells dependent on the antagonistic polycomb repressive complex 2 and its catalytic subunit EZH2, a synthetic-lethal relationship demonstrated in preclinical models and confirmed in patients. The distal (classic) type presents as slow-growing nodules on the hands and forearms of young adults and is often misdiagnosed as a benign lesion; the proximal type, in the pelvis, perineum and trunk, is larger and more aggressive, with rhabdoid features. Both spread to lymph nodes, which is unusual for sarcomas, and to lung.
Localised disease is treated with wide resection and radiotherapy according to soft tissue sarcoma principles; sentinel node evaluation is considered because of nodal spread. Conventional chemotherapy (anthracycline, ifosfamide, gemcitabine-docetaxel) has modest activity. Tazemetostat received FDA accelerated approval in January 2020 for metastatic or locally advanced epithelioid sarcoma not eligible for complete resection, on the basis of the single-arm EZH-202 cohort (objective responses in a minority, but durable, with a benign safety profile). The confirmatory randomised trial EZH-301 adds tazemetostat to doxorubicin in the first line; it was meant to decide whether the accelerated approval would be converted, but Ipsen withdrew the drug in all indications on 9 March 2026 after the SYMPHONY-1 follicular lymphoma trial's data monitoring committee flagged secondary haematologic malignancies (18 of 318 patients, 5.7%, per the FDA safety communication).
Beyond EZH2, checkpoint inhibitors have produced occasional responses (SWI/SNF-deficient tumours are immunogenic in some settings), and combinations of tazemetostat with doxorubicin or immunotherapy are the main directions.
Under one case per million people per year; the distal type peaks in young adults aged 20 to 40, the proximal type in older adults.
Bone sarcomas favour the fast-growing ends of long bones (osteosarcoma) or the shaft (Ewing), soft tissue sarcomas the deep muscle compartments; spread is through the blood to the lungs, rarely via lymph nodes.
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Adamantinoma of bone, Dedifferentiated chordoma, Poorly differentiated chordoma (SMARCB1-deficient), Desmoplastic small round cell tumour, Leiomyosarcoma, Liposarcoma, Synovial sarcoma, Sarcomas (soft tissue, bone, GIST), Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), Chondrosarcoma, Angiosarcoma, Undifferentiated pleomorphic sarcoma (UPS), Myxofibrosarcoma, Alveolar soft part sarcoma, Perivascular epithelioid cell tumour (PEComa), Epithelioid haemangioendothelioma, Malignant peripheral nerve sheath tumour (MPNST), Retroperitoneal sarcoma, Soft tissue sarcoma of the extremity (localised and advanced)
Wide resection with negative margins plus radiotherapy for large, deep or close-margin tumours; lymph node assessment considered because of nodal spread.
Anthracycline-based chemotherapy; tazemetostat (accelerated approval 2020, EZH-202) was withdrawn from all markets in March 2026 after SYMPHONY-1 showed excess secondary blood cancers, so the EZH2 option is gone; clinical trial enrolment encouraged.
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One cancer page on OnCo cites this paper by its DOI; this record gives the citation a page of its own so a reader can follow it without leaving OnCo. Read the abstract above alongside the citing page listed under Related; the record was created automatically from the Europe PMC entry and its figures have not been checked by hand.
One cancer page and one trial page on OnCo cite this paper by its DOI; this record gives the citation a page of its own so a reader can follow it without leaving OnCo. Read the abstract above alongside the citing pages listed under Related; the record was created automatically from the Europe PMC entry and its figures have not been checked by hand.
Query for this cancer: (TITLE:"Epithelioid sarcoma" OR ABSTRACT:"Epithelioid sarcoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Epithelioid sarcoma, not a curated reading list.
Named for its resemblance to carcinoma and granuloma.
Modena and colleagues link the tumour to SWI/SNF deficiency.
Knutson and colleagues, PNAS: EZH2 inhibition kills INI1-negative cells.
FDA, 23 January 2020, based on EZH-202 (Gounder and colleagues, Lancet Oncol 2020).
Doxorubicin with or without tazemetostat, first line (NCT04204941).
Ipsen withdraws tazemetostat in all indications on 9 March 2026 after the SYMPHONY-1 data monitoring committee flagged secondary haematologic malignancies (18/318 patients, 5.7%, per the FDA safety communication).
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Reduce by 50% for bilirubin 20-50 µmol/L and 75% for 50-85 µmol/L.
Alkylating chemotherapy can damage a blood stem cell in a way that shows up years later as myelodysplastic syndrome or acute myeloid leukaemia. It is uncommon, it depends on the total dose, and the risk falls away after about ten years. Knowing the cumulative dose you were given is the single most useful thing on your treatment summary.
Heart damage from cancer treatment: anthracyclines weaken the heart muscle permanently in a dose-related way, trastuzumab does so reversibly, and some kinase inhibitors raise blood pressure or disturb rhythm. Heart function (LVEF) is monitored by ultrasound during treatment.
Hodgkin lymphoma is usually cured, and the problems that follow arrive twenty and thirty years later: heart disease, an underactive thyroid, and second cancers, especially breast cancer in women irradiated to the chest when young. Most of them can be watched for.
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