Primary Cutaneous Adenoid Cystic Carcinoma With Lymph Node Metastasis

Abstract

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.

Primary cutaneous adenoid cystic carcinoma of the upper lid: a case report and literature review

“…It is a neoplasm that is difficult to treat radically, due to its tendency to recur locally, to spread to regional lymph nodes, and to invade perineural spaces [1]. To the best of our knowledge only 7 cases of ACC occurring on the eyelid have been reported in the literature [2][3][4].…”

Axillary lymph node metastases in adenoid cystic carcinoma of the breast. A rare finding

“…However, reports have stated that ACC of other sites (34) have been controlled locally with radiotherapy.…”

Primary cutaneous adenoid cystic carcinoma of the eyelid and literature review

“…Although most commonly observed in the major salivary glands, ACC may also be seen in the minor salivary glands, esophagus, bronchial glands, skin, breast, lungs, vulva, cervix, and prostate (2,3) . ACC is ra rely seen in the palpebral part of the lacrimal gland, and even more ra rely in the eyelids.…”

“…Sentinel lymph node examination has been reported useful for assessment of invasion, as PCACC is known to be locally aggressive and prone to metastasis. In two PCACC cases originating from the scalp and nose, Kato et al detected invasion by sentinel lymph node exa mination (2) . Nevertheless, Cavazza et al were unable to detect invasion using sentinel lymph node examination in their study (1) .…”

“…Adenoid cystic carcinoma (ACC) is an epithelial tumor seen mainly in the major salivary glands and rarely in the minor salivary glands, esophagus, bronchial glands, skin, breast, lungs, vulva, cervix, and pros tate (1)(2)(3) . First described by Boggio et al in 1975, primary cutaneous ACC (PCACC) is a rare type of ACC, with only approximately 60 PCACC cases reported in the literature (4)(5)(6) .…”