Cerebellopontine angle craniopharyngioma in familial adenomatous polyposis

Craniopharyngiomas are benign tumors arising in the sellar and suprasellar regions. Although ectopic tumors do occur, it is usually due to local spread or recurrent tumors. Purely ectopic cerebellopontine angle (CPA) or 4^th ventricle tumors are extremely rare and have been found to be significantly associated with familial adenomatous polyposis (FAP), a genetic disorder.

Only four cases of ectopic CPA craniopharyngioma associated with FAP have been reported to date. Here, we present the 5^th case of ectopic CPA craniopharyngioma on a background of FAP. The previously described cases have been elaborated as well.

CPA tumor with a background of FAP should raise a differential diagnosis of craniopharyngioma, and similarly, a CPA primary ectopic craniopharyngioma may raise suspicion of underlying APC gene mutation.