Pathology · CNS Pathology (Tumors, Degenerative, Infections)

A 9-year-old girl presents with growth failure and bitemporal hemianopia. CT shows a calcified suprasellar cystic mass. Histology would show sheets and cords of squamous epithelium with peripheral palisading, wet keratin, and calcification. The most likely diagnosis is:

  • A Rathke cleft cyst
  • B Papillary craniopharyngioma
  • C Optic nerve glioma
  • D Adamantinomatous craniopharyngioma
Correct answer: D. Adamantinomatous craniopharyngioma

Explanation

Adamantinomatous craniopharyngioma occurs in children, presents with visual field defects, hypothalamic dysfunction and growth failure, and characteristically contains beta-catenin mutations. Its histology includes wet keratin, calcification, and palisading squamous epithelium. Papillary craniopharyngioma occurs almost exclusively in adults, lacks wet keratin and calcification, and carries BRAF V600E mutation, which kills option B.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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