Perinatal atypical teratoid/rhabdoid tumor involving the deep ear structures and complicated by arterial infarction

Atypical teratoid/rhabdoid tumor is a malignant pediatric brain tumor. Unusual invasive behavior of the dura and bony involvement of the deep ear structures and rapid progression in size complicated by arterial infarction have not been described before. A newborn girl presented with increased intracranial pressure. Medical imaging revealed a large mass centered in the left cerebellopontine angle and left middle cranial fossa with large supra-tentorial components associated with destruction of the left petrous bone with involvement of the inner and middle ear structures. Shortly, the tumor rapidly progressed in size and complicated by left middle cerebral artery territory infarction. The patient passed away after a short hospital course. This case report illustrates how rapid and aggressive the natural history of atypical teratoid/thabdoid tumor can be with unusual skull base destruction and deep ear structures involvement.