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.
