The first 60 days: Pheochromocytoma and paraganglioma (PPGL)
Pheochromocytomas and paragangliomas are tumours of adrenaline-producing tissue that cause dangerous blood pressure surges. Surgery after careful blood-pressure blockade cures most, genetic testing finds an inherited cause in nearly half, and for the minority that spread there are now radioactive drugs that home to the tumour and, since 2025, the first oral targeted pill, belzutifan. Below, week by week, is what OnCo's record of Pheochromocytoma and paraganglioma (PPGL) says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- PathologistNamed in the standard of care for: Localised, secreting, All patients.
- RadiologistNamed in the standard of care for: All patients, Metastatic or unresectable.
- SurgeonNamed in the standard of care for: Localised, secreting.
- Medical oncologistNamed in the standard of care for: Metastatic or unresectable.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Metastatic or unresectable.
- Transplant and cell therapy teamNamed in the standard of care for: Metastatic or unresectable.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
- 1.Localised, secretingEndocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors
Alpha-blockade (phenoxybenzamine or doxazosin) for 7 to 14 days, volume expansion, then laparoscopic or open adrenalectomy; cortical-sparing surgery in hereditary bilateral disease.
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.
Germline genetic testing and, for carriers, lifelong biochemical and imaging surveillance; cascade testing of relatives.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- 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?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Adrenal pheochromocytoma, Sympathetic paraganglioma, Head and neckparaganglioma, usually non-secreting.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Localised, secreting
- For my situation (localised, secreting), which of the standard options do you recommend and why?Guideline options include: Alpha-blockade (phenoxybenzamine or doxazosin) for 7 to 14 days, volume expansion, then laparoscopic or open adrenalectomy; cortical-sparing surgery in hereditary bilateral disease.
All patients
- For my situation (all patients), which of the standard options do you recommend and why?Guideline options include: Germline genetic testing and, for carriers, lifelong biochemical and imaging surveillance; cascade testing of relatives.
Metastatic or unresectable
- For my situation (metastatic or unresectable), which of the standard options do you recommend and why?Guideline options include: 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.
- 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?Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Belzutifan, Lutetium-177 dotatate, Peptide receptor radionuclide therapy (PRRT), Sunitinib?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “No reliable predictor of metastasis at diagnosis: molecular classifiers and SDHB status are being validated”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- 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?Open problems are where trials and second opinions matter most.
Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.
- Belzutifan/MK-6482 for the Treatment of Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHLPhase 2 · recruiting · NCT04924075A Phase 2 Study to Evaluate the Efficacy and Safety of Belzutifan (MK-6482, Formerly PT2977) Monotherapy in Participants With Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL) Disease-Associated Tumors, Advanced Gastrointestinal Stromal Tumor (wt GIST), or Advanced Solid Tumors With HIF-2α Related Genetic Alterations
- Study of ONC206 (JZP3507) in Advanced Pheochromocytoma and ParagangliomaPhase 2 · recruiting · NCT07282587A Phase 2 Study of ONC206 in Advanced Pheochromocytoma and Paraganglioma
- Study to Evaluate Safety and Dosimetry of Lutathera in Adolescent Patients With GEP-NETs and PPGLsPhase 2 · active · NCT04711135A Multicenter Open-label Study to Evaluate Safety and Dosimetry of Lutathera in Adolescent Patients With Somatostatin Receptor Positive Gastroenteropancreatic Neuroendocrine (GEP-NET) Tumors, Pheochromocytoma and Paragangliomas (PPGL)
- Targeted Alpha-Particle Therapy for Advanced Somatostatin Receptor Type 2 (SSTR2) Positive TumorsPhase 1/2 · recruiting · NCT05636618A Phase I/IIa First-in-Human Study of [212Pb]VMT-α-NET Targeted Alpha-Particle Therapy for Advanced SSTR2 Positive Tumors
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Pheochromocytoma and paraganglioma (PPGL): the full pagePheochromocytomas and paragangliomas are tumours of adrenaline-producing tissue that cause dangerous blood pressure surges. Surgery after careful blood-pressure blockade cures most, genetic testing finds an inherited cause in nearly half, and for the minority that spread there are now radioactive drugs that home to the tumour and, since 2025, the first oral targeted pill, belzutifan.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- 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.
Every term links to the glossary.