# OnCo record metastatic-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/metastatic-ppgl/>
  a schema:MedicalCondition ;
  onco:kind "cancer" ;
  schema:identifier "metastatic-ppgl" ;
  schema:name "Metastatic pheochromocytoma and paraganglioma"@en ;
  schema:alternateName "Malignant pheochromocytoma"@en, "Metastatic paraganglioma"@en, "Advanced PPGL"@en, "Unresectable pheochromocytoma and paraganglioma"@en ;
  schema:description "Metastatic pheochromocytoma and paraganglioma is disease that has spread to bone, lymph nodes, liver or lungs, the only way these adrenaline-producing tumours are called malignant. It is often slow, so treatment starts with blood pressure control and watching, then moves through radioactive drugs that home to the tumour, the kinase inhibitor sunitinib, chemotherapy and, since 2025, belzutifan."@en ;
  schema:url <https://onco.cc/cancers/metastatic-ppgl/> ;
  schema:dateModified "2026-09-18"^^xsd:date ;
  schema:sameAs <https://en.wikipedia.org/wiki/Pheochromocytoma> ;
  schema:citation <https://doi.org/10.1016/S0140-6736(23)02554-0>, <https://doi.org/10.1210/jc.2014-1498>, <https://en.wikipedia.org/wiki/Pheochromocytoma> ;
  onco:tag "subtype-page", "endocrine" ;
  onco:related <https://onco.cc/cancers/hereditary-ppgl/>, <https://onco.cc/cancers/pheochromocytoma-paraganglioma/>, <https://onco.cc/cancers/advanced-adrenocortical-carcinoma/>, <https://onco.cc/cancers/small-intestinal-net/> ;
  onco:technologies <https://onco.cc/technologies/radioligand-therapy/>, <https://onco.cc/technologies/prrt/>, <https://onco.cc/technologies/mibg-theranostics/>, <https://onco.cc/technologies/sstr-pet/>, <https://onco.cc/technologies/fdg-pet/>, <https://onco.cc/technologies/kinase-inhibitors/>, <https://onco.cc/technologies/cytotoxic-chemotherapy/>, <https://onco.cc/technologies/thermal-ablation/>, <https://onco.cc/technologies/sbrt/>, <https://onco.cc/technologies/active-surveillance/> ;
  onco:targets <https://onco.cc/targets/hif2a/>, <https://onco.cc/targets/sstr2/>, <https://onco.cc/targets/vegf/> ;
  onco:drugs <https://onco.cc/drugs/belzutifan/>, <https://onco.cc/drugs/sunitinib/>, <https://onco.cc/drugs/cabozantinib/>, <https://onco.cc/drugs/lutathera/>, <https://onco.cc/drugs/i131-mibg/>, <https://onco.cc/drugs/temozolomide/>, <https://onco.cc/drugs/cyclophosphamide/>, <https://onco.cc/drugs/vincristine/> ;
  onco:terms <https://onco.cc/terms/adrenalectomy/>, <https://onco.cc/terms/rare-cancers/>, <https://onco.cc/terms/sdh-deficiency/> ;
  onco:trials <https://onco.cc/trials/nct04924075/>, <https://onco.cc/trials/nct07282587/> ;
  onco:keyPapers <https://onco.cc/key-papers/paper-firstmappp-sunitinib-metastatic-ppgl-lancet-2024/>, <https://onco.cc/key-papers/paper-pryma-high-specific-activity-i131-mibg-ppgl-jnm-2019/>, <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/trials/nct07282587/>, <https://onco.cc/drugs/lutathera/>, <https://onco.cc/technologies/prrt/>, <https://onco.cc/drugs/sunitinib/>, <https://onco.cc/drugs/cabozantinib/> ;
  onco:standardOfCare <https://onco.cc/terms/adrenalectomy/>, <https://onco.cc/technologies/sstr-pet/>, <https://onco.cc/technologies/mibg-theranostics/>, <https://onco.cc/technologies/fdg-pet/>, <https://onco.cc/technologies/mri/>, <https://onco.cc/technologies/germline-testing/>, <https://onco.cc/technologies/active-surveillance/>, <https://onco.cc/technologies/sbrt/>, <https://onco.cc/technologies/thermal-ablation/>, <https://onco.cc/technologies/imrt-igrt/>, <https://onco.cc/drugs/lutathera/>, <https://onco.cc/technologies/prrt/>, <https://onco.cc/technologies/radioligand-therapy/>, <https://onco.cc/drugs/i131-mibg/>, <https://onco.cc/technologies/mibg-theranostics/>, <https://onco.cc/drugs/belzutifan/>, <https://onco.cc/drugs/sunitinib/>, <https://onco.cc/drugs/cabozantinib/>, <https://onco.cc/drugs/cyclophosphamide/>, <https://onco.cc/drugs/vincristine/>, <https://onco.cc/drugs/temozolomide/>, <https://onco.cc/technologies/kinase-inhibitors/>, <https://onco.cc/technologies/cytotoxic-chemotherapy/>, <https://onco.cc/trials/nct07282587/>, <https://onco.cc/trials/nct04924075/> .