A 9-year-old boy presents with bitemporal hemianopia and growth failure. CT shows a suprasellar calcified cystic mass. Histology from surgery shows squamous epithelium forming palisaded basal layers, wet keratin, and stellate reticulum. What is the diagnosis?

• A Germinoma
• B Papillary craniopharyngioma
• C Rathke cleft cyst
• D Adamantinomatous craniopharyngioma ✓

Explanation

Adamantinomatous craniopharyngioma occurs in children, presents as a calcified cystic suprasellar mass causing visual field defects and pituitary/hypothalamic dysfunction, and shows palisading squamous epithelium, stellate reticulum, and nodules of anuclear wet keratin. It harbors activating CTNNB1 mutations. Papillary craniopharyngioma occurs in adults, is solid rather than cystic, lacks wet keratin and calcification, and harbors BRAF V600E mutation. Rathke cleft cyst is a non-neoplastic mucin-filled cyst without epithelial stratification.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.