Apocrine Carcinoma of the Perineal Region with Bilateral External Iliac Lymph Node Metastases

A 76-year-old woman noticed a palpable tumor in the perineal region 3 years ago. She visited our hospital due to growth of the tumor for several months. A subcutaneous tumor with a diameter of 2 cm was located anterior to the anus, exposed to the skin and covered with mucus. Imaging revealed that the tumor had invaded the anal sphincter and the vagina, and had metastasized to the bilateral external iliac lymph nodes. A biopsy specimen indicated adenocarcinoma with apocrine differentiation, and the patient was diagnosed with perineal apocrine carcinoma with bilateral external iliac lymphatic metastases. Laparoscopic abdominoperineal resection of the rectum combined with vaginal wall and bilateral external iliac lymphadenectomy was performed. Pathologically, the tumor was found to have invaded to just below the epithelium of the vaginal wall anteriorly and partly to the epithelium of the anal canal posteriorly. Lymph node metastases were found in both external iliac regions. Apocrine adenocarcinoma may have a quiescent period of a few months to several years prior to developing invasive and metastatic potential. Therefore, it is important to make an early diagnosis of this tumor by biopsy.