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WHO Classification of Tumours
Adenoid cystic carcinoma
Skin


Definition

Primary cutaneous adenoid cystic carcinoma (ACC) is a rare, slow-growing adnexal neoplasm characterized by an indolent but progressive course. AAC arises in the dermis, most frequently of the scalp, followed by chest and abdomen, and may extend into the subcutaneous fat. The tumour typically presents as a firm, poorly circumscribed nodule measuring between 0.5 and 8 cm (average 3.2 cm). It is composed of infiltrative nests and cords of basaloid cells arranged in glandular, cystic, cribriform and tubular patterns. The cystic spaces are often filled with mucin. Perineural involvement, a characteristic feature of ACC in other organs, is not as frequently observed in the skin.
Primary cutaneous ACC affects middle-aged and elderly patients (mean age: 58 years) and shows a predilection for women. The tumour recurs in up to 70% of cases; wide surgical excision is therefore recommended

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LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005



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