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18 February 2015 - Case of the Week #343

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Thanks to Dr. Mouna Khmou, University Hospital CHU IBN SINA, Children's Hospital (Morocco), for contributing this case. To contribute a Case of the Week, follow the guidelines on our Case of the Week page.


     

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  • Sternberg's Diagnostic Surgical Pathology Review (2015) by Pier Luigi Di Patre and Darryl Carter. Based on the sixth edition of Sternberg's Diagnostic Surgical Pathology, this multiple choice question and answer resource covers all aspects of Surgical Pathology and is an ideal review tool for board preparation, recertification exams, or just brushing up. Now significantly revised, it includes more than 1,000 questions and answers that emphasize differential diagnostic aspects of problem solving, accompanied by more than 600 full-color illustrations.

For more information, visit our New Books page.

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Case of the Week #343

Clinical History:
A 35 year old woman with no prior medical history consulted for a painless vulvar nodule, first noted 3 years before, which had enlarged over the last few months. There was no history of pain or bleeding. Local and colposcopic examinations revealed a 2 cm freely mobile non-tender nodule in the left labia majora. It was excised under local anesthesia.

Micro images:

H & E images



What is your diagnosis?































Diagnosis:
Hidradenoma papilliferum

Discussion:
Hidradenoma papilliferum of the vulva, see also Skin-Nonmelanocytic Tumor chapter, is also known as papillary hidradenoma, and is the most common benign vulvar adnexal neoplasm (Arch Pathol Lab Med 2013;137:1237). It occurs exclusively in women, typically in the perianal or vulvar regions. Rarely it occurs on the eyelid, nasal area or breast.

Clinically, it is a small, slow growing, firm, red nodule covered by normal skin, often on the labia majora or interlabial folds. It may ulcerate through the skin and simulate carcinoma. Histologically, it resembles intraductal papilloma of the breast, due to its well differentiated papillary fronds in a complex pattern with some stratification and pleomorphism, with ducts lined by apocrine type cells that show decapitation secretion and fibrous stroma. The myoepithelial layer is usually present. Mitoses may be present in both epithelial and myoepithelial cells, but this does not predict an aggressive course (Am J Dermatopathol 2006;28:322). Tumor cells are immunoreactive for AE1/AE3, CK5/6, GCDFP-15, androgen receptor and ER (Adv Anat Pathol 2011;18:1) and negative for S100 and high molecular weight cytokeratin.

The differential diagnosis includes:
Apocrine tubular adenoma: well defined nodule, usually 2 cm or less on the scalp, only rarely on the vulva; has lobular pattern of dermal and subcutaneous tubular apocrine structures with epidermal connection, resembling papillary syringadenoma
Syringocystadenoma papilliferum: warty tumor of the scalp, neck and face that occurs at any age, is associated with nevus sebaceus and basal cell carcinoma; has a glandular papillary pattern with a prominent plasma cell infiltrate.

Simple excision is curative.


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