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Clinical Note
July 19, 2010

Hemangioblastoma of the Cerebellopontine Angle

Author Affiliations

Author Affiliations: Departments of Otolaryngology–Head and Neck Surgery (Drs Bush and Jacob) and Pathology (Dr Ray-Chaudhury) and The Ohio State University College of Medicine (Dr Pritchett), The Ohio State University, Columbus; and Department of Otolaryngology, Wilford Hall Medical Center, Lackland Air Force Base, San Antonio, Texas (Dr Packer). Dr Pritchett is now with the Department of Otolaryngology, University of Michigan, Ann Arbor.

Arch Otolaryngol Head Neck Surg. 2010;136(7):734-738. doi:10.1001/archoto.2010.98

Tumors 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 in 5 CPA lesions are of other etiologies.1,2 Hemangioblastomas (HMBs), which are highly vascular tumors of the central nervous system, represent up to 12% of infratentorial, intracranial tumors in adults and typically arise in the cerebellum, spinal cord, and brainstem.3 One in 4 HMBs is associated with von Hippel Lindau (VHL) disease.4 A cerebellar HMB is found in 44% to 72% of patients with VHL disease5; however, involvement of the CPA is uncommon. 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 HMBs.6,7 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.

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