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Hemangioblastoma of the Cerebellopontine Angle

Hemangioblastoma of the Cerebellopontine Angle CLINICAL NOTE Matthew L. Bush, MD; Cedric Pritchett, MD; Mark Packer, MD; Abhik Ray-Chaudhury, MD; Abraham Jacob, MD umors of the cerebellopontine angle (CPA) constitute 6% to 10% of all intracranial neoplasms. Most are vestibular schwannomas (VSs) and meningiomas, but as many as 1,2 1 in 5 CPA lesions are of other etiologies. Hemangioblastomas (HMBs), which are T highly vascular tumors of the central nervous system, represent up to 12% of infraten- torial, intracranial tumors in adults and typically arise in the cerebellum, spinal cord, and brain- 3 4 stem. One in 4 HMBs is associated with von Hippel Lindau (VHL) disease. A cerebellar HMB is found in 44% to 72% of patients with VHL disease ; however, involvement of the CPA is uncom- mon. When present within the CPA, HMBs may mimic VSs because the 2 entities have similar magnetic resonance imaging (MRI) characteristics. Cystic changes are seen in 70% to 75% of all 6,7 HMBs. We report our experience with a large CPA HMB, which was initially thought to be an atypical cystic VS. Clinical and histologic findings of HMBs are discussed along with the potential for hearing preservation surgery when these lesions occur in the CPA. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA Otolaryngology - Head & Neck Surgery American Medical Association

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Publisher
American Medical Association
Copyright
Copyright 2010 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.
ISSN
2168-6181
eISSN
2168-619X
DOI
10.1001/archoto.2010.98
pmid
20644074
Publisher site
See Article on Publisher Site

Abstract

CLINICAL NOTE Matthew L. Bush, MD; Cedric Pritchett, MD; Mark Packer, MD; Abhik Ray-Chaudhury, MD; Abraham Jacob, MD umors of the cerebellopontine angle (CPA) constitute 6% to 10% of all intracranial neoplasms. Most are vestibular schwannomas (VSs) and meningiomas, but as many as 1,2 1 in 5 CPA lesions are of other etiologies. Hemangioblastomas (HMBs), which are T highly vascular tumors of the central nervous system, represent up to 12% of infraten- torial, intracranial tumors in adults and typically arise in the cerebellum, spinal cord, and brain- 3 4 stem. One in 4 HMBs is associated with von Hippel Lindau (VHL) disease. A cerebellar HMB is found in 44% to 72% of patients with VHL disease ; however, involvement of the CPA is uncom- mon. When present within the CPA, HMBs may mimic VSs because the 2 entities have similar magnetic resonance imaging (MRI) characteristics. Cystic changes are seen in 70% to 75% of all 6,7 HMBs. We report our experience with a large CPA HMB, which was initially thought to be an atypical cystic VS. Clinical and histologic findings of HMBs are discussed along with the potential for hearing preservation surgery when these lesions occur in the CPA.

Journal

JAMA Otolaryngology - Head & Neck SurgeryAmerican Medical Association

Published: Jul 1, 2010

References