Rhabdomyosarcoma of the Temporal Bone: Is Surgical Resection Necessary?

Three patients had embryonal rhabdomyosarcoma of the temporal bone. Their clinical appearances demonstrated the following characteristics: symptoms of an acute process in the middle ear cleft and mastoid, a rapidly growing polypoid mass that was visible in the middle ear and external auditory canal, and seventh nerve involvement and destruction of bone. Definitive treatment with the use of systemic chemotherapy and radiation therapy to the invaded structures was followed by maintenance chemotherapy for up to 24 months. Surgical treatment was sufficient to obtain adequate biopsy material. All patients recovered variable degrees of motor nerve functions. One patient experienced a meningococcal meningitis years after treatment; this condition resulted in total deafness. The results suggest that multiple-drug chemotherapy and radiation therapy for all involved areas are the mainstay of treatment for this disease entity.