Primary intracranial ectopic craniopharyngioma in a patient with probable Gardner's syndrome

J Neurosurg. 2014 Feb;120(2):337-41. doi: 10.3171/2013.10.JNS131401. Epub 2013 Nov 22.

Abstract

The authors describe a patient with an adamantinomatous craniopharyngioma (CPG) arising in the cerebellopontine angle (CPA), who also had probable Gardner's syndrome. This 31-year-old man presented with headache and dizziness. Brain CT and MRI showed a 5 × 4-cm lesion with multiple small calcifications in the left CPA. The patient underwent suboccipital craniotomy with tumor removal. Histopathological findings indicated an adamantinomatous CPG. This patient also showed characteristics of Gardner's syndrome. Although this syndrome is associated with intracranial neoplasms, it is unclear whether patients with both Gardner's syndrome and CPG are part of the heterogeneity of Gardner's syndrome.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adenomatous Polyposis Coli Protein / genetics
  • Adult
  • Brain / diagnostic imaging
  • Brain / pathology
  • Brain Neoplasms / complications
  • Cerebellopontine Angle
  • Craniopharyngioma / complications
  • Craniopharyngioma / genetics
  • Craniopharyngioma / pathology*
  • Craniotomy
  • Follow-Up Studies
  • Gardner Syndrome / complications
  • Gardner Syndrome / genetics
  • Gardner Syndrome / pathology*
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Neurosurgical Procedures
  • Pituitary Neoplasms / complications
  • Pituitary Neoplasms / genetics
  • Pituitary Neoplasms / pathology*
  • Radiosurgery
  • Tomography, X-Ray Computed
  • Treatment Outcome

Substances

  • Adenomatous Polyposis Coli Protein