Vulvar Paget's disease with underlying adenocarcinoma simulating breast carcinoma: case report and review of the literature

Abstract

We report a case of extramammary Paget's disease with underlying adenocarcinoma simulating breast carcinoma of the vulva. An 82-year-old woman was found to have a 5 x 3-cm bulky tumor located in the left labium major, infiltrating to the clitoris, left labium minor, and left lateral tissue of the vulva. Small biopsy of the vulva showed intraepidermal proliferation of Paget cells. The patient underwent wide local excision of the vulvar tumor and dissection of left inguinal lymph nodes. Histopathological examination of the resected specimens revealed that Paget cells were distributed singly or tended to form small nests in the epidermis, and that association of these cells with the underlying carcinoma invading to the subcutis could be seen. The underlying carcinoma was composed of squamoid solid nests with central necrotic debris, mimicking 'comedocarcinoma' of the breast. In other areas, the tumor cells were present in tubular formations and solid cords reminiscent of invasive ductal carcinoma of the breast. Immunohistochemically, the Paget cells and the underlying carcinoma cells were positive for carcinoembryonic antigen, epithelial membrane antigen, estrogen receptors, and glandular keratins except for CK 20. We speculate that our case is vulvar Paget's disease presenting as a manifestation of underlying breast carcinoma of the vulva, which might have arisen from either the ectopic breast tissue or anogenital mammary-like glands.

Invasive ductal carcinoma of the mammary like glands of vulva-with sentinel lymph node identification

“…There was no concomitant Paget's disease in our case which is reported to be seen [4,7,17]. Aggressive prognosis with lymph node metastasis [2,3,[7][8]16] is frequent and bone metastasishas also been reported [10].…”

“…Mammary tissue is described in anogenital region in 1872 by Hartung [1]. Its presence was accepted as ectopic for a long time, and presented as case reports in the literature [2][3][4]. In 1991 van der Putte [5] described 'anogenital sweat glands' with forming lobulus like structures in occasion.…”

“…The clues for the tumor to be primary in the vulva are presence of in situ component [2,8,9]; but it is advised to search for any breast pathology as there can be synchronous or metastatic breast tumor [1]. The treatment regimens differ according to stage of the disease, with wide resection or chemotherapy and be similar to breast carcinoma treatment [2,4,[8][9][10][11][12].…”

Metastatic adenocarcinoma of mammary‐like glands of the vulva successfully treated with surgery and hormonal therapy

“…Recently described cases indicate that this is primarily a condition affecting older women with a mean age at diagnosis of almost 67 years (range 47-87), half of them older than 80. [5][6][7][8][9][10][11][12][13][14][15][16] Only a few patients underwent lymph node staging through inguinofemoral lymphadenectomy, most of them showing lymph node metastases (5/7). We detected pelvic nodal disease upon computed tomography in our patient and consequently performed pelvic lymphadenectomy.…”

Cysts and Epithelial Neoplasms of the Vulva

“…Only a few cases of adenocarcinoma attributed to a histogenesis from the anogenital glands appear in the literature (Abbott & Ahmed 2006), although a few others consider the possibility rather than an origin from 'ectopic breast tissue' (Kennedy et al 1997, Ohira et al 2004, Karakov et al 2005.…”