# Nuclide record hereditary-ppgl (indication). Data CC BY-NC 4.0, attribute "Data from Nuclide (nuclide.cc)". Whole corpus: https://nuclide.cc/api/v1/nuclide.nt
@prefix schema: <https://schema.org/> .
@prefix nuclide: <https://nuclide.cc/ns#> .
@prefix xsd: <http://www.w3.org/2001/XMLSchema#> .

<https://nuclide.cc/indications/hereditary-ppgl/>
  a schema:MedicalCondition ;
  nuclide:kind "indication" ;
  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://nuclide.cc/indications/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> ;
  nuclide:tag "subtype-page", "endocrine" ;
  nuclide:related <https://nuclide.cc/indications/metastatic-ppgl/>, <https://nuclide.cc/indications/pheochromocytoma-paraganglioma/>, <https://nuclide.cc/isotopes/ga-68/>, <https://nuclide.cc/isotopes/lu-177/> ;
  nuclide:technologies <https://nuclide.cc/technologies/sstr-pet/>, <https://nuclide.cc/technologies/radioligand-therapy/>, <https://nuclide.cc/technologies/prrt/>, <https://nuclide.cc/technologies/ct/> ;
  nuclide:targets <https://nuclide.cc/targets/sstr2/> ;
  nuclide:drugs <https://nuclide.cc/drugs/lutathera/> ;
  nuclide:terms <https://nuclide.cc/terms/sdh-deficiency/> ;
  nuclide:pipeline <https://nuclide.cc/drugs/lutathera/>, <https://nuclide.cc/technologies/prrt/> ;
  nuclide:standardOfCare <https://nuclide.cc/terms/sdh-deficiency/>, <https://nuclide.cc/technologies/sstr-pet/>, <https://nuclide.cc/technologies/ct/>, <https://nuclide.cc/drugs/lutathera/>, <https://nuclide.cc/technologies/prrt/> .