The first 60 days: Metastatic pheochromocytoma and paraganglioma
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. Below, week by week, is what OnCo's record of Metastatic pheochromocytoma and paraganglioma 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.
68Ga-DOTATATE PET, 123I-MIBG scintigraphy where 131I-MIBG is available, CT or MRI, FDG-PET for SDHB-related disease; germline testing.
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: Staging, Indolent disease.
- RadiologistNamed in the standard of care for: Staging, Progressive disease, radionuclide therapy.
- SurgeonNamed in the standard of care for: Indolent disease.
- Medical oncologistNamed in the standard of care for: Indolent disease, Progressive disease, radionuclide therapy, Progressive disease, systemic drugs, Trials.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Indolent disease, Progressive disease, radionuclide therapy, Progressive disease, systemic drugs.
- Transplant and cell therapy teamNamed in the standard of care for: Progressive disease, systemic drugs.
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.All patientsEndocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors
Alpha-blockade with beta-blockade added second; metyrosine for refractory symptoms; blockade before every procedure; bone-protective agents for skeletal metastases.
- 2.Indolent diseaseEndocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors
Active surveillance; resection, radiotherapy, thermal ablation or embolisation of dominant or symptomatic lesions.
- 3.Progressive disease, radionuclide therapyEndocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors
Lutetium-177 dotatate for somatostatin-receptor-positive disease; 131I-MIBG for MIBG-avid disease where still available (approved 2018, withdrawn from market 2024).
- 4.Progressive disease, systemic drugsEndocrine Society clinical practice guideline 2014; NCCN Neuroendocrine and Adrenal Tumors
Belzutifan (approved 2025); sunitinib (FIRSTMAPPP); cabozantinib; cyclophosphamide, vincristine and dacarbazine or temozolomide for rapidly progressive or SDHB-related disease.
ONC206, radioligand combinations and next-generation HIF-2 alpha inhibitors.
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 or urinary metanephrines and 3-methoxytyramine, Germline and somatic SDHB, VHL and other cluster status, 68Ga-DOTATATE PET uptake, 123I-MIBG uptake, Rate of progression on serial imaging), 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 Indolent metastatic pheochromocytoma or paraganglioma, Progressive somatostatin-receptor-positive metastatic paraganglioma, MIBG-avid metastatic pheochromocytoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
All patients
- For my situation (all patients), which of the standard options do you recommend and why?Guideline options include: Alpha-blockade with beta-blockade added second; metyrosine for refractory symptoms; blockade before every procedure; bone-protective agents for skeletal metastases.
Staging
- For my situation (staging), which of the standard options do you recommend and why?Guideline options include: 68Ga-DOTATATE PET, 123I-MIBG scintigraphy where 131I-MIBG is available, CT or MRI, FDG-PET for SDHB-related disease; germline testing.
Indolent disease
- For my situation (indolent disease), which of the standard options do you recommend and why?Guideline options include: Active surveillance; resection, radiotherapy, thermal ablation or embolisation of dominant or symptomatic lesions.
Progressive disease, radionuclide therapy
- For my situation (progressive disease, radionuclide therapy), which of the standard options do you recommend and why?Guideline options include: Lutetium-177 dotatate for somatostatin-receptor-positive disease; 131I-MIBG for MIBG-avid disease where still available (approved 2018, withdrawn from market 2024).
- Am I a candidate for Lutetium-177 dotatate, 131I-MIBG (iobenguane I-131) therapy, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Progressive disease, systemic drugs
- For my situation (progressive disease, systemic drugs), which of the standard options do you recommend and why?Guideline options include: Belzutifan (approved 2025); sunitinib (FIRSTMAPPP); cabozantinib; cyclophosphamide, vincristine and dacarbazine or temozolomide for rapidly progressive or SDHB-related disease.
- Am I a candidate for Belzutifan, Sunitinib, Cabozantinib or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Trials
- For my situation (trials), which of the standard options do you recommend and why?Guideline options include: ONC206, radioligand combinations and next-generation HIF-2 alpha inhibitors.
- How do the results of Study of ONC206 (JZP3507) in Advanced Pheochromocytoma and Paraganglioma and Belzutifan/MK-6482 for the Treatment of Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Any stage
- Are there clinical trials I could join, for example of Belzutifan, Belzutifan/MK-6482 for the Treatment of Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL, Study of ONC206 (JZP3507) in Advanced Pheochromocytoma and Paraganglioma, Lutetium-177 dotatate?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 randomised trial has compared radionuclide therapy with drugs or defined their order”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Azedra's withdrawal leaves MIBG-avid, somatostatin-receptor-negative patients without a radionuclide option in many countries”. 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
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Metastatic pheochromocytoma and paraganglioma: the full pageMetastatic 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.
- 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.
- SDH deficiency (SDHB immunohistochemistry loss): Loss of the succinate dehydrogenase enzyme, shown by a negative SDHB stain, marks a small family of tumours (some stomach GISTs, paragangliomas and phaeochromocytomas, a rare kidney cancer) that are often inherited, occur in young people, ignore imatinib and grow slowly; the stain is the trigger for germline testing of the whole family.
- Adrenalectomy: Removing an adrenal gland.
- 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.