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

Michael J. Link, Colin L.W. Driscoll, Caterina Giannini, Allan H. Friedman, E. Sander Connolly, Floyd H. Gilles, Griffith R. Harsh IV

Research output: Contribution to journalArticle

30 Scopus citations

Abstract

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 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.

Original languageEnglish (US)
Pages (from-to)221-226
Number of pages6
JournalNeurosurgery
Volume51
Issue number1
DOIs
StatePublished - Jul 1 2002

Keywords

  • Cerebellopontine angle
  • Craniopharyngioma
  • Familial adenomatous polyposis
  • Gardner syndrome
  • Turcot syndrome

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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    Link, M. J., Driscoll, C. L. W., Giannini, C., Friedman, A. H., Connolly, E. S., Gilles, F. H., & Harsh IV, G. R. (2002). Isolated, giant cerebellopontine angle craniopharyngioma in a patient with Gardner syndrome: Case report. Neurosurgery, 51(1), 221-226. https://doi.org/10.1097/00006123-200207000-00033