11 March 2010 Case of the Week #172
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Thanks to Dr. Semir Vranic, Creighton University Medical Center, Nebraska (USA), for contributing this case. This case was reviewed in May 2020 by Dr. Jennifer Bennett, University of Chicago and Dr. Carlos Parra-Herran, University of Toronto.
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Case of the Week #172
A 73 year old woman presented with a vulvar mass.
What is your diagnosis?
Primary Bartholin's gland adenoid cystic carcinoma
Adenoid cystic carcinomas arise in various sites, including the salivary glands, lacrimal glands, breast, lung and trachea. In the vulva, less than 75 cases have been reported.
Histologically, they show cribriform patterns of cell nests and columns with bland cytologic features, surrounding gland-like spaces with eosinophilic material that is PAS+ diastase resistant (J Surg Oncol 1985;30:132). Perineural and vascular invasion are common. Tumor cells are immunoreactive for low molecular weight keratin, CEA, S100 and type IV collagen (Arch Pathol Lab Med 2007;131:796). Tumor cells have been reported to be immunoreactive for c-kit at other sites (Mod Pathol 2006;19:1351).
The traditional treatment is wide local excision or vulvectomy, with or without nodal dissection. However, surgery may be debilitating and is associated with local recurrence and metastases (Arch Gynecol Obstet 2009;279:747, J Neurosurg 2005;102:543, Eur J Gynaecol Oncol 2009;30:317). As a result, radiation or chemotherapy have been suggested as an alternative (Int J Gynecol Cancer 2007;17:661), although there is no widespread consensus on treatment (Gynecol Oncol 2006;100:422). In the case presented, the patient died a few years later from kidney and liver metastases.
Additional references: Arch Pathol Lab Med 1980;104:523, Eur J Gynaecol Oncol 2009;30:686
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