# OnCo record histiocytoses (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/histiocytoses/>
  a schema:MedicalCondition ;
  onco:kind "cancer" ;
  schema:identifier "histiocytoses" ;
  schema:name "Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms"@en ;
  schema:alternateName "ECD"@en, "Erdheim-Chester disease"@en, "Rosai-Dorfman disease"@en, "RDD"@en, "Rosai-Dorfman-Destombes disease"@en, "Histiocytic sarcoma"@en, "Juvenile xanthogranuloma"@en, "Mixed ECD-LCH"@en, "L-group and R-group histiocytoses"@en ;
  schema:description "Histiocytoses are diseases in which immune scavenger cells build up in bone, heart, brain, kidneys and skin. They used to be treated as inflammatory conditions with steroids and interferon. The discovery that most carry mutations in the same growth pathway as melanoma turned them into targetable cancers: BRAF and MEK inhibitor pills now produce responses in nearly every treated patient."@en ;
  schema:url <https://onco.cc/cancers/histiocytoses/> ;
  schema:dateModified "2026-09-10"^^xsd:date ;
  schema:sameAs <https://en.wikipedia.org/wiki/Erdheim%E2%80%93Chester_disease> ;
  schema:citation <https://www.cancer.gov/types/langerhans>, <https://doi.org/10.1182/blood.2019003507>, <https://doi.org/10.1038/s41591-019-0653-6>, <https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1518> ;
  onco:tag "nci-coverage", "rare", "haematologic", "histiocytosis" ;
  onco:related <https://onco.cc/cancers/langerhans-cell-histiocytosis/>, <https://onco.cc/cancers/melanoma/>, <https://onco.cc/cancers/cmml/> ;
  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/> ;
  onco:targets <https://onco.cc/targets/braf/>, <https://onco.cc/targets/alk/>, <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/> ;
  onco:companies <https://onco.cc/companies/roche-genentech/> ;
  onco:institutions <https://onco.cc/institutions/histiocyte-society/> ;
  onco:pathways <https://onco.cc/pathways/ras-mapk/>, <https://onco.cc/pathways/pi3k-akt-mtor/>, <https://onco.cc/pathways/clonal-haematopoiesis/> ;
  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:bottlenecks <https://onco.cc/bottlenecks/b-rare-cancers/>, <https://onco.cc/bottlenecks/b-generic-repurposing/> ;
  onco:keyPapers <https://onco.cc/key-papers/paper-durham-nat-med/> ;
  onco:pipeline <https://onco.cc/drugs/cobimetinib/>, <https://onco.cc/drugs/vemurafenib/>, <https://onco.cc/drugs/dabrafenib-trametinib/> ;
  onco:standardOfCare <https://onco.cc/drugs/vemurafenib/>, <https://onco.cc/drugs/dabrafenib-trametinib/>, <https://onco.cc/targets/braf/>, <https://onco.cc/drugs/cobimetinib/>, <https://onco.cc/drugs/interferon-alfa/>, <https://onco.cc/drugs/cladribine/>, <https://onco.cc/drugs/cobimetinib/>, <https://onco.cc/drugs/cyclophosphamide/>, <https://onco.cc/drugs/doxorubicin/>, <https://onco.cc/drugs/etoposide/>, <https://onco.cc/drugs/cobimetinib/> .