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5 December 2013 - Case of the Week #293
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Thanks to Dr. Anna Liava (Greece) and Dr. Vasiliki Tzelepi, University of Patras (Greece), for contributing this case and discussion. To contribute a Case of the Week, follow the guidelines on our Case of the Week page.
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Case of the Week #293
A 60 year old woman presented with a slow growing, 2 cm nodule in the left labia majora of the vulva, present for one year, which was biopsied. Mammography revealed no significant findings.
What is your diagnosis?
Adenocarcinoma of the vulva with apocrine features
The tumor consists of irregular glands composed of large polygonal cells with well defined cell membranes, abundant eosinophilic cytoplasm, large hyperchromatic nuclei and prominent nucleoli. Atypical mitotic figures are noted. The tumor cells have a high Ki-67 index (see above), but the cells were negative for GCDFP-15 (not shown). ER and PR were not performed.
Cutaneous vulvar carcinomas are usually squamous cell carcinomas. Primary vulvar adenocarcinomas are rare, with a poorly understood histogenesis. They are classified into (a) extramammary Paget disease, (b) sweat gland carcinoma, and (c) breast-like adenocarcinoma (Pathol Res Pract 2009;205:131), and are distinguished from adenocarcinoma originating in the Bartholin glands (Rare Tumors 2013;5:e40).
Extramamamary Paget disease has large pale staining tumor cells, usually in the lower epidermis, in nests, glandular spaces or continuously along the basement membrane. It may be associated with urothelial carcinoma or anorectal carcinoma. Sweat gland carcinoma derives from native apocrine sweat glands, is usually ER-, PR-, and composed of glandular or papillary cords and tubules, with variable pagetoid components. Breast-like adenocarcinoma may originate from anogenital ER+, PR+ mammary like glands in the interlabial sulci, and is often accompanied by these native glands.
In this case, although ER and PR staining was not done, the absence of GCDFP-15 staining, and the lack of mammary-like glands near the tumor, suggests a sweat gland origin (Am J Dermatopathol 2012;34:773).
Due to its rarity, the prognosis is not well characterized, although regional nodal metastases and distal metastases have been reported.
Nat Pernick, M.D., President
and Palak Thakore, Associate Medical Editor
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