Two patients with primary adenocarcinoma of the middle ear displayed symptoms in the affected ear many years before their condition was diagnosed as adenocarcinoma. Treatment included simple extirpation of the tumor, radical mastoidectomy with or without radiation.
A review of the literature shows 11 cases reported previously. Follow-up of the patients for more than four years revealed four deaths in the reported cases— one from causes unrelated to the tumor and the others showing only local invasion without distant metastases. These tumors grow slowly and have a low-grade of malignancy.