of Week Home
5 December 2013 - Case of the Week #293
All cases are archived on our website. To view them sorted by number, diagnosis or category, visit our Home Page and click on the Case of the Week button on the left hand side. To subscribe or unsubscribe to the Case of the Week or our other email lists, click here.
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.
(1) Don't forget to click on our Amazon.com links on our Home Page and other pages for all your online holiday shopping.
(2) New stains recently added to the Stains and Molecular Markers chapter include
Tissue of origin / unknown primary,
MLL / ALL1,
(3) Good news on the chronic problem of Nature Publishing blocking our website, including the great images from Modern Pathology. Our clever staff have found a way to avoid the block, that will be implemented as we update each of our 6700 topics over the next 1-2 years. Until we get this completed, you can copy and paste the image web address into a new window or tab and press enter.
(4) We have added new Board Review questions, courtesy of BoardVitals, which are accessible via our CME page.
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
30100 Telegraph Road, Suite 408
Bingham Farms, Michigan (USA) 48025
Alternate email: NatPernick@gmail.com