Apocrine carcinoma of the vulva in a band-like arrangement with inflammatory and telangiectatic metastasis via local lymphatic channels.

BACKGROUND: Primary adenocarcinomas of the vulva have been classified as sweat gland carcinomas, extramammary Paget's disease, and primary breast carcinomas of the vulva. They share some common histopathologic features. METHODS: We describe a 72-year-old Japanese woman with apocrine carcinoma of the vulva and local lymphatic metastasis. RESULTS: The patient presented with a bruise on her inguinal area. Physical examination revealed a 4 cm x 7 cm, dark-red, irregularly elevated tumor on the left labium majora. Dome-shaped, flesh-colored, small papulovesicles were scattered on the abdomen, accompanied by erythema and induration. The lesion showed a band-like arrangement. General examination revealed multiple bone metastases, particularly in the spine. Microscopic examination revealed a moderately differentiated adenocarcinoma with signet ring cells. A few pagetoid clear cells were present in the hypertrophic epidermis. The peripheral papulovesicles demonstrated the same histopathologic view as in inflammatory and telangiectatic, metastatic breast carcinoma. Tumor cells were positive for various ductal and glandular markers. Estrogen and progesterone receptors were not expressed. Ultrastructural findings suggested differentiation towards apocrine or mammary glands because of the presence of an apocrine process and electron-dense mucous granules. The patient died in spite of combination chemotherapy and irradiation therapy. CONCLUSIONS: We report a rare case of apocrine carcinoma of the vulva in a band-like arrangement with local lymphatic metastasis which showed the clinical and histopathologic characteristics of inflammatory and telangiectatic carcinoma.