# OnCo record desmoid-tumour (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/desmoid-tumour/>
  a schema:MedicalCondition ;
  onco:kind "cancer" ;
  schema:identifier "desmoid-tumour" ;
  schema:name "Desmoid tumour"@en ;
  schema:alternateName "Aggressive fibromatosis"@en, "Desmoid-type fibromatosis"@en ;
  schema:description "Desmoid tumours are locally aggressive growths of fibroblast-like cells, classed with soft-tissue sarcomas, driven by WNT mutations, that never spread to distant organs but can invade nerves, bowel and muscle. Many stop growing or shrink on their own, so watching first is standard; if they progress, the gamma-secretase inhibitor nirogacestat, approved in 2023, shrinks tumours and relieves pain."@en ;
  schema:url <https://onco.cc/cancers/desmoid-tumour/> ;
  schema:dateModified "2026-09-10"^^xsd:date ;
  schema:sameAs <https://en.wikipedia.org/wiki/Aggressive_fibromatosis> ;
  schema:citation <https://www.cancer.gov/types/soft-tissue-sarcoma>, <https://doi.org/10.1016/j.ejca.2020.06.022>, <https://doi.org/10.1056/NEJMoa2210140>, <https://www.fda.gov/drugs/resources-information-approved-drugs/fda-approves-nirogacestat-desmoid-tumors> ;
  onco:tag "nci-coverage", "rare", "sarcoma" ;
  onco:related <https://onco.cc/cancers/tenosynovial-giant-cell-tumour/>, <https://onco.cc/cancers/dermatofibrosarcoma-protuberans/>, <https://onco.cc/cancers/inflammatory-myofibroblastic-tumour/>, <https://onco.cc/cancers/gist/> ;
  onco:technologies <https://onco.cc/technologies/active-surveillance/>, <https://onco.cc/technologies/mri/>, <https://onco.cc/technologies/cytotoxic-chemotherapy/>, <https://onco.cc/technologies/kinase-inhibitors/> ;
  onco:drugs <https://onco.cc/drugs/nirogacestat/>, <https://onco.cc/drugs/sorafenib/>, <https://onco.cc/drugs/methotrexate/>, <https://onco.cc/drugs/doxorubicin/> ;
  onco:companies <https://onco.cc/companies/springworks/>, <https://onco.cc/companies/bayer/>, <https://onco.cc/companies/elpiscience-suzhou-biopharma/> ;
  onco:pathways <https://onco.cc/pathways/wnt/>, <https://onco.cc/pathways/notch/> ;
  onco:terms <https://onco.cc/terms/rare-cancers/> ;
  onco:trials <https://onco.cc/trials/defi/>, <https://onco.cc/trials/nct04871282/> ;
  onco:bottlenecks <https://onco.cc/bottlenecks/b-rare-cancers/>, <https://onco.cc/bottlenecks/b-toxicity-qol/> ;
  onco:keyPapers <https://onco.cc/key-papers/paper-timbergen-eur-j-cancer/>, <https://onco.cc/key-papers/paper-gounder-n-engl-j-med/> ;
  onco:journals <https://onco.cc/journals/european-journal-of-cancer/> ;
  onco:pipeline <https://onco.cc/drugs/nirogacestat/>, <https://onco.cc/ideas/idea-desmoid-intermittent-dosing/>, <https://onco.cc/trials/defi/>, <https://onco.cc/drugs/varegacestat/> ;
  onco:standardOfCare <https://onco.cc/technologies/active-surveillance/>, <https://onco.cc/technologies/mri/>, <https://onco.cc/drugs/nirogacestat/>, <https://onco.cc/drugs/sorafenib/>, <https://onco.cc/trials/defi/>, <https://onco.cc/drugs/methotrexate/>, <https://onco.cc/technologies/limb-salvage-surgery/> .