OBJECTIVE AND IMPORTANCE: We report the case of a 29-year-old man with Gardner syndrome and an isolated, giant cerebellopontine angle craniopharyngioma.. Our description of this patient is only the second case report of a craniopharyngioma arising primarily in the cerebellopontine angle. CLINICAL PRESENTATION: The patient presented with a 1-year history of progressive neurological impairment and headache. On the basis of the patient's history of multiple dermal fibromas, a cranial osteoma, familial adenomatous polyposis (FAP), a total abdominal colectomy, and an adenoma of the ampulla of Vater, we diagnosed the patient's condition as Gardner syndrome. INTERVENTION: Magnetic resonance imaging showed a large cerebellopontine angle tumor, which was removed through a,suboccipital retromastoid craniotomy. The pathological features were those of an adamantinomatous craniopharyngioma. The patient has done well postoperatively and has no new neurological deficits. A careful retrospective review of the preoparative imaging shows that this tumor was located exclusively in the posterior fossa and was not an extension of a sellar, suprasellar, or. clival craniopharyngioma. CONCLUSION: We present the second reported case of FAP and craniopharyngioma. known genetic link between FAP and craniopharyngioma. Now that the There is no, patient has manifested a primary tumor of the central nervous system with FAP, it is unclear whether he should be classified as having Turcot syndrome. For this patient we recommended vigilant follow-up imaging and forgoing external beam radiotherapy unless there is a documented recurrence-of his craniopharyngioma.
