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Living with cancer· Appointment prep

Appointment sheet: Metastatic pheochromocytoma and paraganglioma

One page to bring and write on: your details, the questions for Metastatic pheochromocytoma and paraganglioma plus your own, the words you may hear, what to bring, the treatments the standard of care names, and room for the answers and agreed next steps. What you type stays in this browser. Print it or save it as a PDF. New to all this? Start with the first 60 days. Orientation, not medical advice.

Tick the questions to print

All of this cancer's questions start ticked. Untick what does not apply; ticks are kept in this browser. .

Your own questions

Shared with the prep pack, so questions you add there appear here too.

Print or save as PDF

Use (or Ctrl+P, Cmd+P on a Mac). To keep a copy, choose Save as PDF as the destination in the print dialog. Only the sheet prints; the controls stay on screen. Your typed notes print where you typed them; empty fields print as ruled lines to write on.

Appointment sheet

Metastatic pheochromocytoma and paraganglioma

Prepared with OnCo (onco.cc/prep/metastatic-ppgl/). Orientation, not medical advice; your team knows your case.

My details

Name
Date of appointment
Hospital and clinician
Who is coming with me

What I know, what is unclear, changes to discuss

Saved in this browser
What I know so far
What is unclear to me
Changes since last time

My questions

18 on the sheet
Newly diagnosed
  1. 1.What is my exact diagnosis, stage, and grade, and which tests established them?
  2. 2.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?
  3. 3.Which subtype is my cancer, and does that change the recommended treatment?
  4. 4.Is germline (inherited) genetic testing recommended for me or my family?
All patients
  1. 5.For my situation (all patients), which of the standard options do you recommend and why?
Staging
  1. 6.For my situation (staging), which of the standard options do you recommend and why?
Indolent disease
  1. 7.For my situation (indolent disease), which of the standard options do you recommend and why?
Progressive disease, radionuclide therapy
  1. 8.For my situation (progressive disease, radionuclide therapy), which of the standard options do you recommend and why?
  2. 9.Am I a candidate for Lutetium-177 dotatate, 131I-MIBG (iobenguane I-131) therapy, and what side effects should I expect?
Progressive disease, systemic drugs
  1. 10.For my situation (progressive disease, systemic drugs), which of the standard options do you recommend and why?
  2. 11.Am I a candidate for Belzutifan, Sunitinib, Cabozantinib or related drugs, and what side effects should I expect?
Trials
  1. 12.For my situation (trials), which of the standard options do you recommend and why?
  2. 13.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?
Any stage
  1. 14.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?
  2. 15.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
  3. 16.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
  4. 17.I read that “No randomised trial has compared radionuclide therapy with drugs or defined their order”. How does that affect my plan?
  5. 18.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?

The words I may hear

  • 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.

Tests and results to bring

Staging: 68Ga-DOTATATE PET, 123I-MIBG scintigraphy where 131I-MIBG is available, CT or MRI, FDG-PET for SDHB-related disease; germline testing.

Biomarker results to ask for: Plasma or urinary metanephrines and 3-methoxytyramine (secretory profile, monitoring), Germline and somatic SDHB, VHL and other cluster status (prognosis, belzutifan and temozolomide sensitivity), 68Ga-DOTATATE PET uptake (lutetium-177 dotatate eligibility), 123I-MIBG uptake (131I-MIBG eligibility), Rate of progression on serial imaging (decides when to treat), Bone scan or FDG-PET for skeletal disease.

Scans and tests linked to this cancer: Active surveillance, FDG PET, Germline (hereditary) testing, MRI, 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

From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.

Answers and next steps

Saved in this browser
What I was told
Agreed next steps, dates and who to call