# OnCo record epithelioid-sarcoma (cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)". Whole corpus: https://onco.cc/api/v1/onco.nt
@prefix schema: <https://schema.org/> .
@prefix onco: <https://onco.cc/ns#> .
@prefix xsd: <http://www.w3.org/2001/XMLSchema#> .

<https://onco.cc/cancers/epithelioid-sarcoma/>
  a schema:MedicalCondition ;
  onco:kind "cancer" ;
  schema:identifier "epithelioid-sarcoma" ;
  schema:name "Epithelioid sarcoma"@en ;
  schema:description "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 The EZH2 inhibitor tazemetostat was approved in 2020 and withdrawn worldwide in March 2026 after secondary blood cancers in a lymphoma trial; the dependency it proved is still a target in development, and chemotherapy remains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."@en ;
  schema:url <https://onco.cc/cancers/epithelioid-sarcoma/> ;
  schema:dateModified "2026-09-10"^^xsd:date ;
  schema:sameAs <https://en.wikipedia.org/wiki/Epithelioid_sarcoma> ;
  schema:citation <https://www.cancer.gov/types/soft-tissue-sarcoma>, <https://doi.org/10.1016/S1470-2045(20)30451-4>, <https://clinicaltrials.gov/study/NCT04204941>, <https://www.globenewswire.com/news-release/2026/03/09/3252192/0/en/update-ipsen-voluntarily-withdraws-tazverik-tazemetostat-in-follicular-lymphoma-and-epithelioid-sarcoma.html>, <https://www.fda.gov/drugs/drug-alerts-and-statements/fda-alerts-health-care-providers-and-patients-about-increased-risk-new-blood-cancers-tazverik>, <https://doi.org/10.1001/jamaoncol.2019.6152>, <https://doi.org/10.1016/S1470-2045(23)00282-6> ;
  onco:tag "nci-coverage", "rare", "sarcoma" ;
  onco:related <https://onco.cc/cancers/atrt/>, <https://onco.cc/cancers/alveolar-soft-part-sarcoma/>, <https://onco.cc/cancers/synovial-sarcoma/>, <https://onco.cc/cancers/pecoma/>, <https://onco.cc/cancers/malignant-peripheral-nerve-sheath-tumour/> ;
  onco:technologies <https://onco.cc/technologies/epigenetic-drugs/>, <https://onco.cc/technologies/limb-salvage-surgery/>, <https://onco.cc/technologies/imrt-igrt/>, <https://onco.cc/technologies/sentinel-node/>, <https://onco.cc/technologies/cytotoxic-chemotherapy/> ;
  onco:targets <https://onco.cc/targets/ezh2/> ;
  onco:drugs <https://onco.cc/drugs/tazemetostat/>, <https://onco.cc/drugs/doxorubicin/>, <https://onco.cc/drugs/ifosfamide/> ;
  onco:companies <https://onco.cc/companies/ipsen/> ;
  onco:pathways <https://onco.cc/pathways/swi-snf-chromatin/>, <https://onco.cc/pathways/epigenetic-reprogramming/> ;
  onco:terms <https://onco.cc/terms/accelerated-approval/>, <https://onco.cc/terms/rare-cancers/> ;
  onco:bottlenecks <https://onco.cc/bottlenecks/b-rare-cancers/> ;
  onco:keyPapers <https://onco.cc/key-papers/paper-gounder-lancet-oncol/>, <https://onco.cc/key-papers/paper-kelly-jama-oncol/>, <https://onco.cc/key-papers/paper-blay-lancet-oncol/> ;
  onco:pipeline <https://onco.cc/trials/tazemetostat-doxorubicin-es/>, <https://onco.cc/drugs/tazemetostat/>, <https://onco.cc/targets/ezh2/>, <https://onco.cc/technologies/epigenetic-drugs/> ;
  onco:standardOfCare <https://onco.cc/technologies/limb-salvage-surgery/>, <https://onco.cc/technologies/imrt-igrt/>, <https://onco.cc/technologies/sentinel-node/>, <https://onco.cc/drugs/tazemetostat/>, <https://onco.cc/drugs/doxorubicin/>, <https://onco.cc/drugs/ifosfamide/> .