Giant Craniopharyngioma

Abstract

Background: Pediatric craniopharyngiomas are adamantinomatous craniopharyngioma. They have solid and cystic parts. They can achieve enormous sizes.

Clinical presentation:  They present with headache, visual disturbances, cognitive decline, hormonal deficiency and short stature.

Key diagnostic features: They are heterogenous suprasellar solid masses with cystic components. The solid part enhances. Calcification is common. They cause sellar erosion.

Differential diagnosis:

Rathke cleft cyst-no solid or enhancing component, calcification is rare, unilocular, the majority are completely or mostly intrasellar.

Pituitary adenoma -usually has intrasellar epicenter with sellar enlargement rather than the suprasellar epicenter, calcification is often absent.

Teratoma - presence of fat is helpful but requires fat-saturated sequences or CT to confirm.

Hypothalamic/optic pathway gliomas-homogenous infiltrating mass which enhances on contrast.

Treatment: Surgical debulking with or without radiotherapy is the mainstay. Recurrence is seen in about 50% patients. Intracystic bleomycin is also useful.