# OnCo record poorly-differentiated-chordoma (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/poorly-differentiated-chordoma/>
  a schema:MedicalCondition ;
  onco:kind "cancer" ;
  schema:identifier "poorly-differentiated-chordoma" ;
  schema:name "Poorly differentiated chordoma (SMARCB1-deficient)"@en ;
  schema:alternateName "SMARCB1-deficient chordoma"@en, "INI1-negative chordoma"@en, "Paediatric poorly differentiated chordoma"@en ;
  schema:description "Poorly differentiated chordoma is a rare, aggressive form of chordoma of children and young adults, mostly at the base of the skull or in the neck, defined by loss of the SMARCB1 (INI1) protein. It grows faster and spreads more than ordinary chordoma, and the SMARCB1 loss makes it a candidate for drugs that block EZH2, though surgery and radiotherapy remain the treatment."@en ;
  schema:url <https://onco.cc/cancers/poorly-differentiated-chordoma/> ;
  schema:dateModified "2026-09-24"^^xsd:date ;
  schema:sameAs <https://en.wikipedia.org/wiki/Chordoma> ;
  schema:citation <https://www.cancer.gov/types/childhood-cancers/patient/rare-childhood-cancers-pdq>, <https://doi.org/10.1007/s00401-016-1574-9>, <https://doi.org/10.1038/s41379-018-0002-1> ;
  onco:tag "subtype-page", "wave4", "rare" ;
  onco:related <https://onco.cc/cancers/chordoma/>, <https://onco.cc/cancers/dedifferentiated-chordoma/>, <https://onco.cc/cancers/atrt/>, <https://onco.cc/cancers/childhood-cancers/> ;
  onco:technologies <https://onco.cc/technologies/proton-therapy/> ;
  onco:targets <https://onco.cc/targets/smarcb1/>, <https://onco.cc/targets/tbxt/> ;
  onco:drugs <https://onco.cc/drugs/tazemetostat/> ;
  onco:standardOfCare <https://onco.cc/cancers/chordoma/>, <https://onco.cc/technologies/proton-therapy/>, <https://onco.cc/drugs/tazemetostat/> .