Primary adenoid cystic carcinoma of Skene's glands

A unique case of a 50-year-old woman is reported who presented with a suburethral mass in the anterior vaginal wall. The resected tumor was an adenoid cystic carcinoma arising from Skene's glands. Tumor was not involving Bartholin's glands. In addition to the typical glands and areas with cysts containing periodic acid-Schiff positive intraluminal material, less well-differentiated areas with neoplastic cells growing in a trabecular or solid pattern were also seen. Prominent perineural invasion was noted throughout the tumor. Immunostaining revealed positive reactions for cytokeratins, carcinoembryonic antigen, and focally for S-100 protein. Ultrastructural studies showed epithelial cells with well-formed basal lamina and prominent microvilli. This case illustrates an uncommon site, the Skene's glands for adenoid cystic carcinoma in the female genitourinary tract.