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Clinical Note |

Hemangioblastoma of the Cerebellopontine Angle

Matthew L. Bush, MD; Cedric Pritchett, MD; Mark Packer, MD; Abhik Ray-Chaudhury, MD; Abraham Jacob, MD
Arch Otolaryngol Head Neck Surg. 2010;136(7):734-738. doi:10.1001/archoto.2010.98.
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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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Figure 1.

A T1-weighted axial magnetic resonance image with gadolinium at the level of the internal auditory canal showing enhancement of the left cerebellopontine angle solid tumor along its capsule and septations. Note that the tumor involves the medial 4 mm of the canal.

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Figure 2.

A T2-weighted axial magnetic resonance image demonstrating intratumoral hyperintensity as well as highlighting the cystic portion of this left cerebellopontine angle tumor.

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Figure 3.

A T1-weighted axial magnetic resonance imaging fluid-attenuated inversion-recovery sequence demonstrating the cerebellar brain parenchymal edema and compression of the fourth ventricle.

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Figure 4.

Photomicrographs of the neoplasm. A, Low-power view showing a highly vascular neoplasm consisting of capillaries and small vascular channels scattered among neoplastic cells (hematoxylin-eosin, original magnification ×100). B, High-power view demonstrating stromal cells with atypical hyperchromatic nuclei and copious lipid-laden cytoplasm (hematoxylin-eosin, original magnification ×400).

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