Abstract
Epithelioid angiosarcoma (EA) is a rare, highly malignant tumor that affects mainly the deep soft tissues of the extremities and has a poor prognosis. This angiosarcoma occurs rarely in the middle ear. Herein, we describe an unusual case of 36-year-old man who presented with an 8-year history of progressive left-sided hearing loss and unbearable otalgia for 1-month. Otoscopic examination showed that the left external auditory canal was dry and the tympanic membrane was intacted. An audiogram demonstrated a moderate mixed hearing loss in the left ear. High-resolution computed tomography of the temporal bone demonstrated a huge destructive lesion with significant bone erosion, a round soft tissue mass located mainly in the left mastoid cavity measuring 3.0 cm × 3.4 cm × 3.1 cm, and some soft tissue among the rest of the mastoid air cells and tympanic cavity. The appearance of the lesion did not change with contrast enhancement. Magnetic resonance imaging showed that the soft mass had mixed signal intensity with heterogeneous enhancement on T1- and T2-weighted images and well-defined margins. Histopathology and immunohistochemistry revealed an EA. To our knowledge, this is the first reported case of an EA with clear boundaries located mainly in the mastoid cavity.
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