Pheochromocytoma and paraganglioma (PPGL)
Prepared with OnCo (onco.cc/prep/pheochromocytoma-paraganglioma/). Orientation, not medical advice; your team knows your case.
My details
What I know, what is unclear, changes to discuss
Saved in this browserMy questions
13 on the sheet- 1.What is my exact diagnosis, stage, and grade, and which tests established them?
- 2.Which biomarkers have been tested on my tumour (for example Plasma free or urinary fractionated metanephrines, Germline panel testing, SDHB immunohistochemistry, 68Ga-DOTATATE PET, 123I-MIBG scintigraphy), and what were the results?
- 3.Which subtype is my cancer, and does that change the recommended treatment?
- 4.Is germline (inherited) genetic testing recommended for me or my family?
- 5.For my situation (localised, secreting), which of the standard options do you recommend and why?
- 6.For my situation (all patients), which of the standard options do you recommend and why?
- 7.For my situation (metastatic or unresectable), which of the standard options do you recommend and why?
- 8.Am I a candidate for Belzutifan, Lutetium-177 dotatate, 131I-MIBG (iobenguane I-131) therapy or related drugs, and what side effects should I expect?
- 9.Are there clinical trials I could join, for example of Belzutifan, Lutetium-177 dotatate, Peptide receptor radionuclide therapy (PRRT), Sunitinib?
- 10.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 11.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 12.I read that “No reliable predictor of metastasis at diagnosis: molecular classifiers and SDHB status are being validated”. How does that affect my plan?
- 13.I read that “Withdrawal of 131I-MIBG from the market left a gap that 177Lu-DOTATATE and alpha-emitters are filling”. How does that affect my plan?
The words I may hear
- Adrenalectomy: Removing an adrenal gland.
- Hereditary cancer syndromes: About 5-10% of cancers arise from an inherited gene fault.
- Rare cancers: Rare cancers are those with fewer than about 6 new cases per 100,000 people per year.
Tests and results to bring
Biomarker results to ask for: Plasma free or urinary fractionated metanephrines, Germline panel testing (SDHA/B/C/D, SDHAF2, VHL, RET, NF1, MAX, TMEM127, FH, EPAS1), SDHB immunohistochemistry (loss indicates SDHx), 68Ga-DOTATATE PET (SSTR2 expression; selects for PRRT), 123I-MIBG scintigraphy (selects for 131I-MIBG), Tumour size, extra-adrenal location and SDHB status as metastatic risk factors.
Scans and tests linked to this cancer: Germline (hereditary) testing, Somatostatin receptor PET (68Ga/64Cu-DOTATATE), MIBG imaging and 131I-MIBG therapy.
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- Localised, secreting: Alpha-blockade (phenoxybenzamine or doxazosin) for 7 to 14 days, volume expansion, then laparoscopic or open adrenalectomy; cortical-sparing surgery in hereditary bilateral disease. (Adrenalectomy, Germline (hereditary) testing)
- Metastatic or unresectable: Belzutifan (FDA May 2025, LITESPARK-015); 177Lu-DOTATATE for SSTR-positive disease; 131I-MIBG where available; sunitinib (FIRSTMAPPP); CVD or temozolomide chemotherapy for rapidly progressive or SDHB-mutant disease; alpha-blockade throughout. (Belzutifan, Lutetium-177 dotatate, Peptide receptor radionuclide therapy (PRRT), 131I-MIBG (iobenguane I-131) therapy, MIBG imaging and 131I-MIBG therapy, Sunitinib, Temozolomide, Cyclophosphamide, Vincristine)
- All patients: Germline genetic testing and, for carriers, lifelong biochemical and imaging surveillance; cascade testing of relatives. (Germline (hereditary) testing, Hereditary cancer syndromes)
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.