Isolated, giant cerebellopontine angle craniopharyngioma in a patient with Gardner syndrome: case report.

Journal Article (Journal Article)

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 patients 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 preoperative 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. There is no known genetic link between FAP and craniopharyngioma. Now that the 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.

Full Text

Duke Authors

Cited Authors

  • Link, MJ; Driscoll, CLW; Giannini, C

Published Date

  • July 2002

Published In

Volume / Issue

  • 51 / 1

Start / End Page

  • 221 - 225

PubMed ID

  • 12182421

International Standard Serial Number (ISSN)

  • 0148-396X

Digital Object Identifier (DOI)

  • 10.1097/00006123-200207000-00033


  • eng

Conference Location

  • United States