Primary adenoid cystic carcinoma of the middle ear and mastoid.

Two primary adenoid cystic carcinomas of the middle ear and mastoid are presented. This pathologic condition is uncommon and no large series exist in the literature. Adenoid cystic carcinoma could arise from ectopic salivary gland tissue located in the wall of the jugular bulb in proximity to the hypotympanum. These tumors demonstrate much invasion of bone and soft tissue. The surgical rationale in the management of this rare tumor is discussed.