Question

A child presents with visual disturbances and delayed growth. Imaging reveals a suprasellar mass, and histopathology shows wet keratin compact, eosinophilic anucleate keratin. What is the most likely diagnosis?

• A. Medulloblastoma
• B. Rathke pouch cyst
• C. Pituitary adenoma
• D. Craniopharyngioma

Hint:

Child with suprasellar mass, visual symptoms, endocrine problems, and wet keratin = craniopharyngioma.

Answer 1

The Correct Option is D

Step 1: Understanding the Question:
A suprasellar mass in a child causing visual (optic chiasm compression) and endocrine (growth delay) issues is most commonly a Craniopharyngioma.
Step 2: Detailed Explanation:

Craniopharyngioma: These are benign but locally aggressive tumors derived from remnants of Rathke’s pouch. They exhibit a bimodal age distribution (children and adults over 50).

Adamantinomatous Type (Pediatric): This is the variant seen in children. Histologically, it is characterized by:
1. Wet Keratin: Lumps of lamellated, eosinophilic, anucleate keratin.
2. Palisading Epithelium: Peripheral layer of columnar cells.
3. Machine Oil Fluid: The cyst contains thick, cholesterol-rich brown fluid.

Radiology: These tumors are often cystic and highly likely to show calcification on CT scans.

Clinical Impact: Pressure on the optic chiasm causes bitemporal hemianopsia; pressure on the pituitary/hypothalamus causes growth hormone deficiency and diabetes insipidus.

Step 3: Final Answer:
The specific description of "wet keratin" in a pediatric suprasellar mass is pathognomonic for an Adamantinomatous Craniopharyngioma.