Otopathology in Angiosarcoma of the Temporal Bone.

ABSTRACT

OBJECTIVES/HYPOTHESIS: Investigate the otopathology of angiosarcoma of the temporal bone, which has not been previously described in the literature. STUDY DESIGN: Postmortem evaluation and literature review. METHODS: Postmortem histological evaluation of the temporal bones and review of the literature for the treatment and prognosis of this rare disease were performed. RESULTS: A 50-year-old male with right chronic otitis media presented with progressive hearing loss, disequilibrium, otalgia, and acute facial paresis. Biopsy of the external auditory canal was unrevealing, but specimens from a canal wall down tympanomastoidectomy later showed high-grade angiosarcoma. Magnetic resonance imaging demonstrated an unresectable middle ear and mastoid mass extending superiorly into the temporal lobe. The patient received induction chemotherapy followed by proton beam radiation therapy and concurrent paclitaxel and bevacizumab. His course was complicated by a cerebrospinal fluid leak and cauda equina syndrome from leptomeningeal sarcomatosis. The patient died after developing meningitis and a temporal lobe abscess. Postmortem otopathology revealed persistent angiosarcoma in the internal auditory canal, although none was found in the middle ear or mastoid. There was inflammatory infiltrate throughout the mastoid, with direct extension of neutrophils and bacteria into the cochlea and through the tegmen into the middle cranial fossa. CONCLUSIONS: Angiosarcoma of the temporal bone can arise in the setting of chronic otitis media. In this case, postmortem temporal bone sections demonstrated viable cancer despite chemoradiation. Inflammatory infiltrates crossing from the middle ear/mastoid into the labyrinth and central nervous system illustrate pathways for the development of otogenic meningitis. LEVEL OF EVIDENCE 4 Laryngoscope, 129737-742, 2019.