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

Case report

Myoung Soo Kim M.D., Ph.D.1, You Sun Kim M.D., Ph.D.2, Hye Kyung Lee M.D., Ph.D.3, Ghi Jai Lee M.D., Ph.D.4, Chan Young Choi M.D., Ph.D.5, and Chae Heuck Lee M.D., Ph.D.5
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  • 1 Departments of Neurosurgery,
  • | 2 Internal Medicine,
  • | 3 Pathology, and
  • | 4 Diagnostic Radiology, Seoul Paik Hospital, Inje University College of Medicine, Seoul; and
  • | 5 Department of Neurosurgery, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea
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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.

Abbreviations used in this paper:

APC = adenomatous polyposis coli; CPA = cerebellopontine angle; CPG = craniopharyngioma; FAP = familial adenomatous polyposis; GSK-3β = glycogen synthase kinase–3β.

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Contributor Notes

Address correspondence to: Myoung Soo Kim, M.D., Department of Neurosurgery, Seoul Paik Hospital, Inje University College of Medicine, 85 Jeo-dong 2-ga, Jung-gu, Seoul, Korea 100-032. email: hanibalkms@hanmail.net.

Please include this information when citing this paper: published online November 22, 2013; DOI: 10.3171/2013.10.JNS131401.

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