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.
Bush ML, Pritchett C, Packer M, Ray-Chaudhury A, Jacob A. Hemangioblastoma of the Cerebellopontine Angle. Arch Otolaryngol Head Neck Surg. 2010;136(7):734–738. doi:10.1001/archoto.2010.98
Artificial Intelligence Resource Center
Customize your JAMA Network experience by selecting one or more topics from the list below.