Adenoid cystic carcinoma of ear
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Synopsis

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.
Codes
ICD10CM:C44.201 – Unspecified malignant neoplasm of skin of unspecified ear and external auricular canal
SNOMEDCT:
11671000 – Adenoid cystic carcinoma
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Differential Diagnosis & Pitfalls
- Squamous cell carcinoma
- Basal cell carcinoma
- Cholesteatoma
- Infected atheroma
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Last Reviewed:06/14/2022
Last Updated:07/07/2022
Last Updated:07/07/2022