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Cancers
Hereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127)
Data

Cancers·Hereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127)

DataHereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127)

Back to the Hereditary pheochromocytoma and paraganglioma hub →26 connected records

Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.

Overview What it is Finding it Treating it Evidence The science Where you are Living with it What is coming Data

Data

Related pages

26

Direct links plus the targets, companies, and technologies of this cancer's products.

Similar pages

not linked directly; found by shared links

Cancer
Pancreatic neuroendocrine tumours
Shares Belzutifan/MK-6482 for the Treatment of Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL, Somatostatin receptor PET (68Ga/64Cu-DOTATATE), HIF-2α, Peptide receptor radionuclide therapy (PRRT) and the tags subtype-page, endocrine.
Cancer
Advanced and metastatic adrenocortical carcinoma (ENSAT stage IV or unresectable)
Shares Adrenalectomy, Localised adrenocortical carcinoma (ENSAT stage I to III, resectable), Metastatic pheochromocytoma and paraganglioma, SBRT / SABR (stereotactic radiotherapy) and the tags subtype-page, endocrine.
Cancer
Grade 3 well-differentiated neuroendocrine tumour
Shares Somatostatin receptor PET (68Ga/64Cu-DOTATATE), Peptide receptor radionuclide therapy (PRRT), Somatostatin receptor 2, Lutetium-177 dotatate and the tags subtype-page, endocrine.
Cancer
Small intestinal neuroendocrine tumours
Shares Somatostatin receptor PET (68Ga/64Cu-DOTATATE), Metastatic pheochromocytoma and paraganglioma, Peptide receptor radionuclide therapy (PRRT), Somatostatin receptor 2 and the tags subtype-page, endocrine.
Cancer
Extrapulmonary neuroendocrine carcinoma
Shares Somatostatin receptor PET (68Ga/64Cu-DOTATATE), Lutetium-177 dotatate, CT (computed tomography) and the tags subtype-page, endocrine.
Cancer
Lung neuroendocrine tumours (typical and atypical carcinoid)
Shares Somatostatin receptor PET (68Ga/64Cu-DOTATATE), Peptide receptor radionuclide therapy (PRRT), Somatostatin receptor 2, Lutetium-177 dotatate and the tags subtype-page, endocrine.
Cancer
Esthesioneuroblastoma (olfactory neuroblastoma)
Shares Somatostatin receptor PET (68Ga/64Cu-DOTATATE), Peptide receptor radionuclide therapy (PRRT), Rare cancers, Somatostatin receptor 2 and the tag subtype-page.
Cancer
Epithelioid haemangioendothelioma
Shares Active surveillance, CT (computed tomography), MRI, IMRT / IGRT (modern external beam) and the tag subtype-page.

technologies

11

Active surveillance CT (computed tomography)Germline (hereditary) testing IMRT / IGRT (modern external beam)MRI Peptide receptor radionuclide therapy (PRRT)Radioligand therapy (beta emitters)Robotic & minimally invasive surgery SBRT / SABR (stereotactic radiotherapy)Small-molecule kinase inhibitors Somatostatin receptor PET (68Ga/64Cu-DOTATATE)

targets

3

HIF-2α RET Somatostatin receptor 2

drugs

2

ApprovedSmall-molecule HIF-2α inhibitor

Belzutifan · Welireg

ApprovedRadioligand therapy (beta)

Lutetium-177 dotatate · Lutathera

companies

2

Merck & Co. (MSD)Novartis

terms

4

Adrenalectomy Hereditary cancer syndromes Rare cancers SDH deficiency (SDHB immunohistochemistry loss)

trials

1

Belzutifan/MK-6482 for the Treatment of Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL

key papers

3

Belzutifan for renal cell carcinoma and other tumours in von Hippel-Lindau disease Germline mutations in nonsyndromic pheochromocytoma Pheochromocytoma and paraganglioma: an Endocrine Society clinical practice guideline

Machine-readable versions

The same record for scripts and assistants; checked 2026-09-18. Data CC BY-NC 4.0, attribute “Data from OnCo (onco.cc)”.

JSON with neighbours Markdown context RDF Turtle Sections with routes and counts API root

Not yet reviewedNeeds expert (clinical) and patient advocate review. Review this page or see the review queue and roster.

Model panel commentary is coming to this page: machine commentary, not clinical review.

ProvenanceLast edited 2026-09-18 · Jude Gomila · Key papers for the 39 rare-cancer subtype pages · diff · file

Wikipedia

Sources & links

Endocrine Society PPGL guideline 2014
Belzutifan in VHL (NEJM 2021)
Wikipedia

subtype-page endocrine

Data

JSON · ·

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MCP server: npx -y onco-mcp, tools search, get_entity, list_kind, ask, context, compare. This record is get_entity or context with id hereditary-ppgl.

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