# OnCo record rosai-dorfman-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/rosai-dorfman-disease/>
  a schema:MedicalCondition ;
  onco:kind "cancer" ;
  schema:identifier "rosai-dorfman-disease" ;
  schema:name "Rosai-Dorfman-Destombes disease"@en ;
  schema:alternateName "Rosai-Dorfman disease"@en, "RDD"@en, "Sinus histiocytosis with massive lymphadenopathy"@en, "R-group histiocytosis"@en, "Destombes-Rosai-Dorfman disease"@en ;
  schema:description "Rosai-Dorfman disease is a rare histiocytosis in which large immune cells called histiocytes fill the neck lymph nodes or grow in the skin, bones, nose, brain coverings or kidneys. Many cases fade without treatment, so it is watched unless it threatens an organ, when surgery, steroids, sirolimus or, for the third with a growth-pathway mutation, MEK inhibitors such as cobimetinib are used."@en ;
  schema:url <https://onco.cc/cancers/rosai-dorfman-disease/> ;
  schema:dateModified "2026-09-18"^^xsd:date ;
  schema:sameAs <https://en.wikipedia.org/wiki/Rosai%E2%80%93Dorfman_disease> ;
  schema:citation <https://doi.org/10.1182/blood-2018-03-839753>, <https://en.wikipedia.org/wiki/Rosai%E2%80%93Dorfman_disease> ;
  onco:tag "subtype-page", "haematologic" ;
  onco:related <https://onco.cc/cancers/erdheim-chester-disease/>, <https://onco.cc/cancers/histiocytoses/>, <https://onco.cc/cancers/lch-single-system/> ;
  onco:technologies <https://onco.cc/technologies/fdg-pet/>, <https://onco.cc/technologies/cgp/>, <https://onco.cc/technologies/mri/>, <https://onco.cc/technologies/histopathology-ihc/>, <https://onco.cc/technologies/kinase-inhibitors/>, <https://onco.cc/technologies/active-surveillance/>, <https://onco.cc/technologies/imrt-igrt/> ;
  onco:targets <https://onco.cc/targets/braf/> ;
  onco:drugs <https://onco.cc/drugs/cobimetinib/>, <https://onco.cc/drugs/cladribine/>, <https://onco.cc/drugs/methotrexate/>, <https://onco.cc/drugs/rituximab/> ;
  onco:terms <https://onco.cc/terms/driver-mutation/>, <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-rosai-dorfman-destombes-consensus-recommendations-blood-2018/>, <https://onco.cc/key-papers/paper-emile-revised-classification-of-histiocytoses-blood-2016/> ;
  onco:pipeline <https://onco.cc/drugs/cobimetinib/> ;
  onco:standardOfCare <https://onco.cc/technologies/histopathology-ihc/>, <https://onco.cc/technologies/cgp/>, <https://onco.cc/technologies/fdg-pet/>, <https://onco.cc/technologies/mri/>, <https://onco.cc/technologies/active-surveillance/>, <https://onco.cc/technologies/imrt-igrt/>, <https://onco.cc/drugs/cladribine/>, <https://onco.cc/drugs/methotrexate/>, <https://onco.cc/drugs/rituximab/>, <https://onco.cc/drugs/cobimetinib/>, <https://onco.cc/pathways/ras-mapk/>, <https://onco.cc/technologies/kinase-inhibitors/> .