# 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 The EZH2 inhibitor tazemetostat, the first epigenetic drug approved for a solid tumour, was approved in 2020 and withdrawn worldwide in March 2026 after secondary blood cancers in a lymphoma trial, so there is no targeted drug today."@en ;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/> .