# OnCo record erdheim-chester-disease (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/erdheim-chester-disease/>
  a schema:MedicalCondition ;
  onco:kind "cancer" ;
  schema:identifier "erdheim-chester-disease" ;
  schema:name "Erdheim-Chester disease"@en ;
  schema:alternateName "ECD"@en, "Polyostotic sclerosing histiocytosis"@en, "L-group histiocytosis (adult)"@en, "BRAF-mutant histiocytosis"@en ;
  schema:description "Erdheim-Chester disease is a rare histiocytosis, a cancer-like overgrowth of immune cells called histiocytes that scar the long bones, the tissue around the kidneys and heart, the brain and the skin. Most cases carry the BRAF V600E mutation or another fault in the same growth pathway, and the melanoma drugs vemurafenib and cobimetinib now control the disease in most patients."@en ;
  schema:url <https://onco.cc/cancers/erdheim-chester-disease/> ;
  schema:dateModified "2026-09-18"^^xsd:date ;
  schema:sameAs <https://en.wikipedia.org/wiki/Erdheim%E2%80%93Chester_disease> ;
  schema:citation <https://doi.org/10.1182/blood.2019003507>, <https://doi.org/10.1038/s41586-019-1012-y>, <https://en.wikipedia.org/wiki/Erdheim%E2%80%93Chester_disease> ;
  onco:tag "subtype-page", "haematologic" ;
  onco:related <https://onco.cc/cancers/rosai-dorfman-disease/>, <https://onco.cc/cancers/histiocytoses/>, <https://onco.cc/cancers/lch-multisystem/>, <https://onco.cc/cancers/braf-v600-melanoma/> ;
  onco:technologies <https://onco.cc/technologies/kinase-inhibitors/>, <https://onco.cc/technologies/fdg-pet/>, <https://onco.cc/technologies/liquid-biopsy/>, <https://onco.cc/technologies/cgp/>, <https://onco.cc/technologies/mri/>, <https://onco.cc/technologies/active-surveillance/> ;
  onco:targets <https://onco.cc/targets/braf/>, <https://onco.cc/targets/pik3ca/> ;
  onco:drugs <https://onco.cc/drugs/vemurafenib/>, <https://onco.cc/drugs/cobimetinib/>, <https://onco.cc/drugs/dabrafenib-trametinib/>, <https://onco.cc/drugs/interferon-alfa/>, <https://onco.cc/drugs/cladribine/>, <https://onco.cc/drugs/methotrexate/> ;
  onco:terms <https://onco.cc/terms/braf-v600-mutation/>, <https://onco.cc/terms/driver-mutation/>, <https://onco.cc/terms/ctdna/>, <https://onco.cc/terms/retroperitoneum/> ;
  onco:keyPapers <https://onco.cc/key-papers/paper-who-2022-myeloid-khoury-leukemia-2022/>, <https://onco.cc/key-papers/paper-ve-basket-vemurafenib-erdheim-chester-lch-jama-oncol-2018/>, <https://onco.cc/key-papers/paper-erdheim-chester-disease-consensus-recommendations-blood-2020/>, <https://onco.cc/key-papers/paper-emile-revised-classification-of-histiocytoses-blood-2016/> ;
  onco:pipeline <https://onco.cc/drugs/cobimetinib/>, <https://onco.cc/drugs/vemurafenib/>, <https://onco.cc/drugs/dabrafenib-trametinib/> ;
  onco:standardOfCare <https://onco.cc/terms/braf-v600-mutation/>, <https://onco.cc/technologies/cgp/>, <https://onco.cc/technologies/fdg-pet/>, <https://onco.cc/technologies/mri/>, <https://onco.cc/technologies/liquid-biopsy/>, <https://onco.cc/drugs/vemurafenib/>, <https://onco.cc/drugs/dabrafenib-trametinib/>, <https://onco.cc/drugs/cobimetinib/>, <https://onco.cc/targets/braf/>, <https://onco.cc/technologies/kinase-inhibitors/>, <https://onco.cc/drugs/cobimetinib/>, <https://onco.cc/pathways/ras-mapk/>, <https://onco.cc/technologies/kinase-inhibitors/>, <https://onco.cc/drugs/interferon-alfa/>, <https://onco.cc/drugs/cladribine/>, <https://onco.cc/drugs/methotrexate/>, <https://onco.cc/technologies/fdg-pet/>, <https://onco.cc/technologies/active-surveillance/> .