Adenoid cystic carcinoma (ACC) of the ear is a malignant tumor arising from secretory glandular cells. It is extremely rare in the auditory canal and has a high misdiagnosis rate, which allows for its slow progression. It is the most common ceruminous gland malignancy. Long-term fatality ranges from 60%- 90%.

Pathology demonstrates 3 types of growth patterns. These include the cribriform (nest) pattern, the tubular (cordlike) pattern, and the basaloid (solid) pattern. ACC mainly affects the salivary glands, but may also primarily involve the lacrimal gland, the larynx, the breast, and the lungs.

Adenoid cystic carcinomas grow along neurons. Lymph node metastases are unusual, but late, distant metastasis to the lungs is expected. Hematogenous tumor spread is characteristic.

Women are slightly more affected than men. ACC typically occurs between the ages of 30-50 years. Poor prognostic factors include increased age, Black race, male sex, and distant / lymph node metastasis.

Early symptoms include an ear canal mass, otalgia, hearing changes, and serous / clear otorrhea. Recurrent otitis externa, bleeding, and neuropathy occur later. The average tumor size is about 1.6 cm.