A 7-year-old girl presents with short stature, visual field defects, and diabetes insipidus. MRI shows a suprasellar cystic and solid mass containing coarse calcification. The solid component is composed of nests of squamous epithelium with peripheral palisading, wet keratin, and nuclear beta-catenin accumulation. What is the diagnosis?

• A Optic pathway pilocytic astrocytoma
• B Rathke cleft cyst
• C Adamantinomatous craniopharyngioma ✓
• D Papillary craniopharyngioma

Explanation

Adamantinomatous craniopharyngioma affects children, forms calcified cystic suprasellar masses, and is driven by activating CTNNB1 mutations producing nuclear beta-catenin; wet keratin and palisaded epithelium are characteristic. Papillary craniopharyngioma occurs in adults, lacks calcification, and harbours BRAF V600E mutation instead. Rathke cleft cyst contains mucinous fluid without epithelial nests or calcification, and optic glioma is glial rather than epithelial, so these distractors fail on both imaging and histology.

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.