The association of ectopic craniopharyngioma in the fourth ventricle with familial adenomatous polyposis: illustrative case

Abstract

BACKGROUND Craniopharyngioma (CP) often arises in the sellar and suprasellar areas; ectopic CP in the posterior fossa is rare. Familial adenomatous polyposis (FAP) is a genetic disorder involving the formation of numerous adenomatous polyps in the gastrointestinal tract, and it is associated with other extraintestinal manifestations. OBSERVATIONS The authors reported the case of a 63-year-old woman with FAP who presented with headache and harbored a growing mass in the fourth ventricle. Magnetic resonance imaging (MRI) findings revealed a well-circumscribed mass with high intensity on T1-weighted images and low intensity on T2-weighted images and exhibited no contrast enhancement. Gross total resection was performed and histopathology revealed an adamantinomatous CP (aCP). The authors also reviewed the previous reports of ectopic CP in the posterior fossa and found a high percentage of FAP cases among the ectopic CP group, thus suggesting a possible association between the two diseases. LESSONS An ectopic CP may be reasonably included in the differential diagnosis in patients with FAP who present with well-circumscribed tumors in the posterior fossa.

Isolated fourth ventricle craniopharyngioma: Representative case illustration and review of literature

“…Obstructing the 4 th ventricle can give rise to hydrocephalus, and the patient may develop rapid deterioration of the consciousness level with other signs of raised intracranial pressure. [ 1 , 2 , 3 , 6 , 9 , 11 , 12 ]…”

“…Obstructing the 4 th ventricle can give rise to hydrocephalus, and the patient may develop rapid deterioration of the consciousness level with other signs of raised intracranial pressure. [1,2,3,6,9,11,12] Management Gross total resection was the surgical technique that was most frequently employed. However, Bashir et al achieved near-total…”

“…Bashir et al [3] [1] 2018 24/M None H/A, double vision ere is no evidence of recurrence at two years follow-up Uemura et al [12] No recurrence at the one-year follow-up M: Male, F: Female, H/A: Headache, FM: Foramen Magnum, GTR: Gross total resection, NTR: Near total resection removal of the tumor due to dense adherence of the cyst wall to the brainstem. [3] In this instance, our tumor was devoid of cystic components and not adherent with surrounding neurovascular structures, which facilitated easy peeling of the tumor.…”

“…They often start in the suprasellar region of the brain’s infundibulohypophysial axis and spread to affect the hypothalamus, optic chiasm, cranial nerves, third ventricle, and blood arteries. [ 1 , 12 ]…”

“…ey often start in the suprasellar region of the brain's infundibulohypophysial axis and spread to affect the hypothalamus, optic chiasm, cranial nerves, third ventricle, and blood arteries. [1,12] Although few craniopharyngiomas are seen in the infrasellar, intrasellar, or anterior regions, the tumor is often suprasellar in position. ere are two possible types of posterior fossa craniopharyngiomas: recurrence/extension and the exceedingly uncommon initial tumor (de novo).…”