The first 60 days: Craniopharyngioma
Craniopharyngioma is a benign but destructive tumour growing from embryonic remnants beside the pituitary gland and hypothalamus. Surgery, or limited surgery plus radiotherapy, cures most people, but the price can be lifelong hormone deficiency and severe obesity. The adult (papillary) form carries a BRAF mutation and shrinks dramatically with BRAF and MEK inhibitors, its first drug treatment. Below, week by week, is what OnCo's record of Craniopharyngioma 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.
Gross total resection (transsphenoidal or craniotomy) with endocrine replacement; observation with serial MRI.
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.
- RadiologistNamed in the standard of care for: Newly diagnosed, no or limited hypothalamic involvement.
- SurgeonNamed in the standard of care for: Newly diagnosed, no or limited hypothalamic involvement, Hypothalamic involvement, Papillary craniopharyngioma, BRAF V600E.
- Medical oncologistNamed in the standard of care for: Newly diagnosed, no or limited hypothalamic involvement, Hypothalamic involvement, Papillary craniopharyngioma, BRAF V600E.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Hypothalamic involvement, Papillary craniopharyngioma, BRAF V600E.
- Palliative and supportive care teamNamed in the standard of care for: Survivorship.
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.
Hypothalamus-sparing subtotal resection or cyst drainage followed by conformal or proton radiotherapy; intracystic therapy for predominantly cystic tumours.
BRAF plus MEK inhibition (vemurafenib-cobimetinib per Alliance A071601, or dabrafenib-trametinib) before or instead of extensive surgery; radiotherapy after response.
Lifelong endocrinology follow-up, management of hypothalamic obesity, sleep and behavioural sequelae; structured survivorship care.
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 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?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 Adamantinomatous, Papillary.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Newly diagnosed, no or limited hypothalamic involvement
- For my situation (newly diagnosed, no or limited hypothalamic involvement), which of the standard options do you recommend and why?Guideline options include: Gross total resection (transsphenoidal or craniotomy) with endocrine replacement; observation with serial MRI.
Hypothalamic involvement
- For my situation (hypothalamic involvement), which of the standard options do you recommend and why?Guideline options include: Hypothalamus-sparing subtotal resection or cyst drainage followed by conformal or proton radiotherapy; intracystic therapy for predominantly cystic tumours.
Papillary craniopharyngioma, BRAF V600E
- For my situation (papillary craniopharyngioma, braf v600e), which of the standard options do you recommend and why?Guideline options include: BRAF plus MEK inhibition (vemurafenib-cobimetinib per Alliance A071601, or dabrafenib-trametinib) before or instead of extensive surgery; radiotherapy after response.
- Am I a candidate for Dabrafenib + trametinib, Vemurafenib, Cobimetinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Survivorship
- For my situation (survivorship), which of the standard options do you recommend and why?Guideline options include: Lifelong endocrinology follow-up, management of hypothalamic obesity, sleep and behavioural sequelae; structured survivorship care.
Any stage
- Are there clinical trials I could join, for example of Dabrafenib + trametinib, Proton therapy, GLP-1 receptor agonists and obesity-related cancer risk?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 “Hypothalamic obesity in survivors has no reliable treatment; GLP-1 agonists, setmelanotide and oxytocin are in trials”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- 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?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.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Craniopharyngioma: the full pageCraniopharyngioma is a benign but destructive tumour growing from embryonic remnants beside the pituitary gland and hypothalamus. Surgery, or limited surgery plus radiotherapy, cures most people, but the price can be lifelong hormone deficiency and severe obesity. The adult (papillary) form carries a BRAF mutation and shrinks dramatically with BRAF and MEK inhibitors, its first drug treatment.
- 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.
- 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.
Every term links to the glossary.