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Craniopharyngioma is a benign, partly cystic epithelial tumor of the sellar region, presumed to derive from Rathke pouch epithelium per WHO-adapted descriptions catalogued by Orphanet (Orphanet:54595). Two clinicopathological forms are recognized: adamantinomatous craniopharyngioma, which affects primarily children and young adults, and papillary craniopharyngioma. Estimated prevalence is 1–9 in 100,000 per Orphanet.
Phenotypic data were not populated in the assembled knowledge packet for craniopharyngioma; the catalogued phenotype count for this disease entry is zero. The WHO-adapted Orphanet definition characterizes the condition by its sellar region tumor origin rather than by enumerated clinical symptom profiles.
Craniopharyngioma is considered to arise from Rathke pouch epithelium, per WHO-adapted Orphanet-linked definitions. No causative germline genes are recorded in the known_genes field for this disease. No Mendelian inheritance pattern is catalogued. The condition presents typically as sporadic in origin.
Diagnostic classification distinguishes two clinicopathological subtypes: adamantinomatous and papillary craniopharyngioma. Tumor size is a recognized prognostic factor per the WHO-adapted Orphanet definition, with lesions exceeding 5 cm in diameter associated with markedly worse outcomes. No specific diagnostic laboratory criteria are enumerated in the current packet.
No FDA-approved pharmacological treatments for craniopharyngioma are catalogued in the current packet. Active and recruiting clinical trials listed in ClinicalTrials.gov document investigations of surgical resection extent, proton radiotherapy approaches, targeted molecular agents for mutation-defined tumor subtypes, and biologic interventions for the adamantinomatous subtype. Both pediatric and adult disease cohorts are represented in the trial registry.
24 trials found
Per the WHO-adapted definition catalogued in Orphanet sources, the extent of surgical resection is identified as the most significant factor associated with disease recurrence. Lesions exceeding 5 cm in diameter carry a markedly worse prognosis. A patient registry is maintained by the Raymond A. Wood Foundation, catalogued in the packet's patient organization field.
Multiple clinical trials catalogued in ClinicalTrials.gov address craniopharyngioma, including Phase 2 studies investigating both the adamantinomatous and papillary subtypes, as well as imaging-focused and observational research. Kisho's publication pipeline has classified 69 items in this disease area, the majority of which are review articles and meta-analyses.
Data assembled from 4 of 12 sources · Last updated Sep 19, 2026, 1:03 PM UTC
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AI-curated news mentioning craniopharyngioma
Updated May 11, 2026
The OUTSPREAD study investigates the effects of growth hormone treatment on linear growth and metabolic outcomes in patients with childhood-onset craniopharyngioma. Findings contribute to understanding treatment impacts in this rare disease population.