Loading
Craniopharyngioma is a benign but destructive brain 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 markedly with BRAF and MEK inhibitors, its first drug treatment.
Craniopharyngioma is a WHO grade 1 epithelial tumour of the sellar and suprasellar region with two distinct types. Adamantinomatous craniopharyngioma (ACP), the childhood form, carries activating CTNNB1 (beta-catenin) mutations, forms cysts filled with motor-oil fluid, and invades the hypothalamus; papillary craniopharyngioma (PCP), almost exclusively adult, carries BRAF V600E in nearly every case. Neither metastasises, but both damage vision, pituitary function and the hypothalamic centres that control appetite, sleep and temperature.
Management has shifted from radical resection at any cost to preserving the hypothalamus. Gross total resection cures if achieved, but attempts to strip tumour from the hypothalamus cause hypothalamic obesity, which is refractory to diet and exercise and is the dominant determinant of quality of life in survivors. Hypothalamus-sparing subtotal resection followed by conformal or proton radiotherapy gives equivalent tumour control with fewer severe late effects, and is now the favoured approach for tumours with hypothalamic involvement (KRANIOPHARYNGEOM 2007 and St Jude data). Cysts can be managed with catheter drainage, intracystic interferon or bleomycin, or stereotactic radiosurgery. Lifelong endocrine replacement is the norm.
The molecular findings created two therapeutic openings. In papillary tumours, BRAF plus MEK inhibition (vemurafenib-cobimetinib in the Alliance A071601 phase 2, Lancet Oncology 2024) produced marked shrinkage in almost all treated patients, allowing surgery and radiotherapy to be reduced; dabrafenib-trametinib case series show the same. In adamantinomatous tumours, the inflammatory cyst fluid is rich in IL-6, and the IL-6 receptor antibody tocilizumab has shrunk cysts in children in case series and an early trial. Treatments for hypothalamic obesity itself (GLP-1 agonists, setmelanotide, oxytocin) are being studied.
Rare: a few percent of childhood brain tumours, with a second peak in adults in their fifties and sixties (NCI PDQ).
Gliomas infiltrate along white matter and can cross the corpus callosum, medulloblastoma sits in the cerebellum, and CNS lymphoma favours deep periventricular tissue; none spread through lymph nodes.
No conventional lymphatics: gliomas spread along white matter tracts and, rarely, through cerebrospinal fluid; medulloblastoma can seed the spine.
Same organ: Lactotroph pituitary neuroendocrine tumour (prolactinoma), Somatotroph pituitary neuroendocrine tumour (acromegaly), Corticotroph pituitary neuroendocrine tumour (Cushing disease and silent corticotroph tumour), Gonadotroph pituitary neuroendocrine tumour (non-functioning adenoma), Thyrotroph pituitary neuroendocrine tumour (TSH-secreting), Pineocytoma and pineal parenchymal tumour of intermediate differentiation, Pineoblastoma, Papillary tumour of the pineal region, Choroid plexus carcinoma, Glioma & glioblastoma, Primary CNS lymphoma, Medulloblastoma, Paediatric low-grade glioma, Diffuse midline glioma, H3 K27-altered (including DIPG), Atypical teratoid/rhabdoid tumour (ATRT), Ependymoma, Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma, Brain and spinal cord tumours (all types), Astrocytoma, IDH-mutant (grades 2 to 4), Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, Paediatric high-grade glioma (excluding diffuse midline glioma), Meningioma, Brain metastases (secondary brain tumours), Vestibular schwannoma (acoustic neuroma), Central nervous system germ cell tumours (germinoma and non-germinomatous), Spinal cord tumours (intramedullary and intradural), WNT-activated medulloblastoma, SHH-activated medulloblastoma, Group 3 and group 4 medulloblastoma (non-WNT/non-SHH)
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Gross total resection (transsphenoidal or craniotomy) with endocrine replacement; observation with serial MRI.
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.
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
For survivors of craniopharyngioma and other hypothalamic injuries, whose weight gain has resisted diet and conventional drugs, this is the first randomised evidence of a treatment that works on the damaged satiety pathway itself. The FDA extended the Imcivree label to acquired hypothalamic obesity in March 2026 on this trial.
One cancer page on OnCo cites this paper by its DOI; this record gives the citation a page of its own so a reader can follow it without leaving OnCo. Read the abstract above alongside the citing page listed under Related; the record was created automatically from the Europe PMC entry and its figures have not been checked by hand.
Query for this cancer: (TITLE:"Craniopharyngioma" OR ABSTRACT:"Craniopharyngioma" OR TITLE:"Adamantinomatous craniopharyngioma" OR ABSTRACT:"Adamantinomatous craniopharyngioma" OR TITLE:"Papillary craniopharyngioma" OR ABSTRACT:"Papillary craniopharyngioma" OR TITLE:"Childhood craniopharyngioma" OR ABSTRACT:"Childhood craniopharyngioma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Craniopharyngioma, not a curated reading list.
Brastianos and colleagues (Nature Genetics) show near-universal BRAF V600E in PCP and CTNNB1 in ACP.
Case report (JNCI) leads to the Alliance A071601 trial.
KRANIOPHARYNGEOM 2007 shows radical resection with hypothalamic injury worsens quality of life without improving control.
Vemurafenib plus cobimetinib produces responses in nearly all BRAF V600E papillary craniopharyngiomas (Lancet Oncology).
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Dabrafenib: take on an empty stomach. Trametinib: take on an empty stomach; both cause pyrexia.
Severe photosensitivity: sun protection.
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
See all on the product pages:CobimetinibDabrafenib + trametinibVemurafenib·Printable cards in the navigator
Newly diagnosed? Read the first 60 days with Craniopharyngioma, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.