# OnCo record hereditary-ppgl (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/hereditary-ppgl/>
  a schema:MedicalCondition ;
  onco:kind "cancer" ;
  schema:identifier "hereditary-ppgl" ;
  schema:name "Hereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127)"@en ;
  schema:alternateName "Familial paraganglioma syndromes"@en, "SDHB-related paraganglioma"@en, "SDHD-related head and neck paraganglioma"@en, "VHL-associated pheochromocytoma"@en, "MEN2-associated pheochromocytoma"@en, "Hereditary PPGL"@en ;
  schema:description "Hereditary pheochromocytoma and paraganglioma is the inherited form of these adrenaline-producing tumours, caused by a fault in one of more than a dozen genes, most often SDHB, SDHD, VHL and RET. Knowing the gene changes care: SDHB carriers have the highest risk of spread, VHL and MEN2 patients get adrenal-sparing surgery because tumours arise on both sides, and relatives are screened."@en ;
  schema:url <https://onco.cc/cancers/hereditary-ppgl/> ;
  schema:dateModified "2026-09-18"^^xsd:date ;
  schema:sameAs <https://en.wikipedia.org/wiki/Paraganglioma> ;
  schema:citation <https://doi.org/10.1210/jc.2014-1498>, <https://doi.org/10.1056/NEJMoa2103425>, <https://en.wikipedia.org/wiki/Paraganglioma> ;
  onco:tag "subtype-page", "endocrine" ;
  onco:related <https://onco.cc/cancers/metastatic-ppgl/>, <https://onco.cc/cancers/pheochromocytoma-paraganglioma/>, <https://onco.cc/cancers/medullary-thyroid-cancer/>, <https://onco.cc/cancers/localised-adrenocortical-carcinoma/> ;
  onco:technologies <https://onco.cc/technologies/germline-testing/>, <https://onco.cc/technologies/sstr-pet/>, <https://onco.cc/technologies/radioligand-therapy/>, <https://onco.cc/technologies/prrt/>, <https://onco.cc/technologies/mri/>, <https://onco.cc/technologies/ct/>, <https://onco.cc/technologies/active-surveillance/>, <https://onco.cc/technologies/imrt-igrt/>, <https://onco.cc/technologies/sbrt/> ;
  onco:targets <https://onco.cc/targets/hif2a/>, <https://onco.cc/targets/sstr2/>, <https://onco.cc/targets/ret/> ;
  onco:drugs <https://onco.cc/drugs/belzutifan/>, <https://onco.cc/drugs/lutathera/> ;
  onco:terms <https://onco.cc/terms/hereditary-cancer-syndromes/>, <https://onco.cc/terms/adrenalectomy/>, <https://onco.cc/terms/sdh-deficiency/>, <https://onco.cc/terms/rare-cancers/> ;
  onco:trials <https://onco.cc/trials/nct04924075/> ;
  onco:keyPapers <https://onco.cc/key-papers/paper-belzutifan-vhl-jonasch-nejm-2021/>, <https://onco.cc/key-papers/paper-neumann-germline-mutations-nonsyndromic-pheochromocytoma-nejm-2002/>, <https://onco.cc/key-papers/paper-endocrine-society-pheochromocytoma-paraganglioma-guideline-jcem-2014/> ;
  onco:pipeline <https://onco.cc/drugs/belzutifan/>, <https://onco.cc/trials/nct04924075/>, <https://onco.cc/drugs/lutathera/>, <https://onco.cc/technologies/prrt/>, <https://onco.cc/technologies/germline-testing/> ;
  onco:standardOfCare <https://onco.cc/technologies/germline-testing/>, <https://onco.cc/terms/hereditary-cancer-syndromes/>, <https://onco.cc/terms/sdh-deficiency/>, <https://onco.cc/technologies/sstr-pet/>, <https://onco.cc/technologies/mri/>, <https://onco.cc/technologies/ct/>, <https://onco.cc/terms/adrenalectomy/>, <https://onco.cc/technologies/robotic-surgery/>, <https://onco.cc/technologies/active-surveillance/>, <https://onco.cc/technologies/imrt-igrt/>, <https://onco.cc/technologies/sbrt/>, <https://onco.cc/technologies/mri/>, <https://onco.cc/technologies/germline-testing/>, <https://onco.cc/drugs/belzutifan/>, <https://onco.cc/trials/nct04924075/>, <https://onco.cc/drugs/lutathera/>, <https://onco.cc/technologies/prrt/> .