Multiple Meningiomas in a Patient with Cowden Syndrome

Margaret Pain; Armine Darbinyan; Mary Fowkes; Raj Shrivastava

doi:10.1055/s-0036-1584265

Journal of Neurological Surgery Reports, Inhaltsverzeichnis

J Neurol Surg Rep 2016; 77(03): e128-e133
DOI: 10.1055/s-0036-1584265

Case Report

Georg Thieme Verlag KG Stuttgart · New York

Multiple Meningiomas in a Patient with Cowden Syndrome

Autor*innen

Margaret Pain

¹Department of Neurosurgery, The Mount Sinai Hospital, New York, New York, United States
Armine Darbinyan

²Department of Pathology, The Mount Sinai Hospital, New York, New York, United States
Mary Fowkes

²Department of Pathology, The Mount Sinai Hospital, New York, New York, United States
Raj Shrivastava

¹Department of Neurosurgery, The Mount Sinai Hospital, New York, New York, United States

Abstract

Background Cowden syndrome is a rare, multisystem disease manifesting with increased hamartomas and neoplasms. Though meningioma has been documented in patients with Cowden syndrome, the relationship between these two phenomena is still unclear.

Case Description We report a case of a 43-year-old female patient with a known PTEN mutation and clinical history of Cowden syndrome. A workup of headache demonstrated two skull base meningiomas. At the time of surgery, several additional tiny meningiomas were detected in the same region.

Conclusions The development of multiple meningiomas in a patient with predisposition for tumor is more than coincidental. Though PTEN mutations and deletions have not been shown to be critical for meningioma development, this case challenges that conclusion. In light of recent genetic advances in meningioma molecular pathogenesis, the role of the PTEN/AKT/PI3K pathway is discussed.

Keywords

Cowden syndrome - PTEN - meningioma

Volltext

Referenzen

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