Coexistence of intracranial arteriovenous malformation with Turcot's syndrome: a case report

Surg Neurol. 1999 Oct;52(4):397-9. doi: 10.1016/s0090-3019(99)00099-3.

Abstract

Turcot's syndrome is a combination of multiple adenomatous gastrointestinal polyps and neuroepithelial tumors of the central nervous system (CNS). A 16-year-old man presented with Turcot's syndrome consisting of nonfamilial colorectal polyposis and multiple fibrillary astrocytomas accompanied by ruptured intracranial arteriovenous malformation (AVM) manifesting as generalized epileptic activity. Multiple CNS tumors are extremely unusual in Turcot's syndrome. This is the first description of Turcot's syndrome associated with an intracranial AVM. This case may correspond to a new phenotypic variant of colonic polyposis syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adenomatous Polyposis Coli / complications*
  • Adenomatous Polyposis Coli / diagnostic imaging
  • Adenomatous Polyposis Coli / pathology
  • Adolescent
  • Astrocytoma / complications*
  • Astrocytoma / diagnostic imaging
  • Brain Neoplasms / complications*
  • Brain Neoplasms / diagnostic imaging
  • Cerebral Hemorrhage / complications*
  • Cerebral Hemorrhage / diagnostic imaging
  • Cerebral Hemorrhage / etiology
  • Colonoscopy
  • Fatal Outcome
  • Humans
  • Intracranial Arteriovenous Malformations / complications*
  • Intracranial Arteriovenous Malformations / diagnostic imaging
  • Male
  • Syndrome
  • Tomography, X-Ray Computed