Craniopharyngioma
Prepared with OnCo (onco.cc/prep/craniopharyngioma/). 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
14 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 CTNNB1 exon 3 mutation and nuclear beta-catenin, BRAF V600E, Hypothalamic involvement grade on MRI, Pituitary hormone panel and visual fields at baseline, Body-mass-index trajectory after treatment), 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 (newly diagnosed, no or limited hypothalamic involvement), which of the standard options do you recommend and why?
- 6.For my situation (hypothalamic involvement), which of the standard options do you recommend and why?
- 7.For my situation (papillary craniopharyngioma, braf v600e), which of the standard options do you recommend and why?
- 8.Am I a candidate for Dabrafenib + trametinib, Vemurafenib, Cobimetinib, and what side effects should I expect?
- 9.For my situation (survivorship), which of the standard options do you recommend and why?
- 10.Are there clinical trials I could join, for example of Dabrafenib + trametinib, Proton therapy, GLP-1 receptor agonists and obesity-related cancer risk?
- 11.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 12.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 13.I read that “Hypothalamic obesity in survivors has no reliable treatment; GLP-1 agonists, setmelanotide and oxytocin are in trials”. How does that affect my plan?
- 14.I read that “No systemic therapy is established for adamantinomatous tumours; IL-6 blockade and MEK inhibition (downstream of the beta-catenin-driven inflammatory programme) are being tested”. How does that affect my plan?
The words I may hear
- Late effects and survivorship toxicity: Health problems appearing months or decades after treatment ends: heart damage, infertility, second cancers, lymphoedema, dry mouth, memory problems, weak bones.
Tests and results to bring
Newly diagnosed, no or limited hypothalamic involvement: Gross total resection (transsphenoidal or craniotomy) with endocrine replacement; observation with serial MRI.
Biomarker results to ask for: CTNNB1 exon 3 mutation and nuclear beta-catenin (adamantinomatous), BRAF V600E (papillary), Hypothalamic involvement grade on MRI (Puget or Muller grading), Pituitary hormone panel and visual fields at baseline, Body-mass-index trajectory after treatment.
Scans and tests linked to this cancer: MRI.
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- Hypothalamic involvement: Hypothalamus-sparing subtotal resection or cyst drainage followed by conformal or proton radiotherapy; intracystic therapy for predominantly cystic tumours. (Proton therapy, IMRT / IGRT (modern external beam))
- Papillary craniopharyngioma, BRAF V600E: BRAF plus MEK inhibition (vemurafenib-cobimetinib per Alliance A071601, or dabrafenib-trametinib) before or instead of extensive surgery; radiotherapy after response. (Dabrafenib + trametinib, Vemurafenib, Cobimetinib)
- Survivorship: Lifelong endocrinology follow-up, management of hypothalamic obesity, sleep and behavioural sequelae; structured survivorship care. (Survivorship care and late-effects surveillance)
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.