Primary adenocarcinoma of the vulva, possibly cloacogenic : a report of two cases

: Primary adenocarcinoma of the vulva, arising in continuity with the epidermis and unrelated to the underlying mucus glands, is a distinctly uncommon neoplasm. The origin of such an unusual tumor, although attributed to cloacal remnants, remains speculative. We describe two patients with this rare lesion. No other primary tumor was found in either of the two after a thorough investigation. One patient was treated with radical vulvectomy and was free of disease for 10 years, until her death from unrelated causes. The second patient was treated with wide local excision and was free of disease four years postoperatively. Our experience indicates the need to recognize this tumor as a primary and not metastatic neoplasm of the vulva. We also noticed that in both cases, despite its histologic malignancy and ulceration, the tumor was superficial in its extent and without any tendency for deeper invasion. It can therefore be treated effectively with wide local excision, thus eliminating the need for radical vulvectomy.