Angiosarcoma of the Temporal Bone

Objective: The objectives of this report are: (1) to present a case of a primary temporal bone angiosarcoma, a rare entity with only 8 reported cases in the literature; and (2) to present a literature review and discussion of the clinical presentation, diagnosis and management of options for this challenging disease.

Introduction: Angiosarcoma is a rare high-grade malignant tumor with endothelial differentiation that can involve soft tissue or bone. The majority of bone angiosarcomas occur in the limbs, with less frequent occurrence in the ribs, pelvis, and vertebra. The skull is an extremely unusual site, and only eight cases of temporal bone angiosarcoma have been described in the literature.

Case Presentation: A 56-year-old woman presented with acute altered mental status. Her history was notable for 6 months of progressively worsening left neck pain and headache. For the month preceding her presentation, she was getting progressively more ataxic. Initial examination revealed a non-tender subcutaneous 2-cm mass in the left mastoid region, with no overlying skin changes. Left otoscopy showed opacification of the superior aspect of the tympanic membrane by a voluminous mass. A CT of the head with contrast revealed a heterogeneously enhancing left mastoid mass eroding the inner table and sigmoid sinus plate. There was a large extracalvarial soft tissue component to the mass, with significant mass effect on the fourth ventricle causing moderate hydrocephalus. An MRI of the head revealed a complex enhancing mass in the left posterior fossa with extracranial extension causing significant mass effect on the left cerebellum. An MR venogram showed lack of enhancement of the distal aspect of the left transverse sinus, sigmoid sinus, and jugular vein. Histological examination revealed solid nests and large sheets of spindle cells interspersed between vessels. Immunohistochemistry showed a strong cytoplasmic staining of tumor cells for vimentin-, CD31-, and factor VIII-related antigen. A final diagnosis of angiosarcoma was made. Imaging workup for a possible extracranial primary tumor was negative. The patient was deemed not a surgical candidate, and was ultimately managed with chemoradiation.

Conclusion: This report presents a rare case of a primary temporal bone angiosarcoma, with review of the literature regarding the clinical presentation, diagnostic workup, and management options for this challenging pathologic entity.