Primary cutaneous adenoid cystic carcinoma with lymph node metastasis

We report on a case of primary cutaneous adenoid cystic carcinoma with local recurrence and lymph node metastasis. The patient was a 52-year-old Japanese woman. The initial cutaneous lesion was a skin-colored, centrally elevated, thumb-sized nodule with hair loss on the scalp in the right side of the parietal region. Three and half years after the initial combined treatment consisting of surgery and irradiation, a recurrent nodule on the left side of the parietal region of the scalp and a lymph node metastasis with perinodal tissue involvement in the left retroauricular area were discovered. In the literature on primary cutaneous adenoid cystic carcinoma, the average age in the 37 cases reported, including the present case, is 58.1 years, and 23 of the patients are women. Thirty-two percent of the carcinomas (12 cases) occur on the scalp and 16% (6 cases) on the breast. The average size of the tumors in the 24 documented cases is 3.2 cm. The local recurrence rate is 51% (19 cases). Distant metastasis to the lungs and pleura are recorded in three cases. Lymph node metastasis is recorded only in two cases including the present case. Multiple modalities including surgery, irradiation, and chemotherapy should be used for this locally aggressive and potentially metastasizing carcinoma.