Paget's Disease of the Vulva Detected in Vulvar and Vaginal Brushing Smears A Case Report

Background Vulvar Paget's disease comprises only 1-8% of malignant vulvar tumors. It is associated with an underlying carcinoma in 20-30% of cases. Clinically, it manifests as erythematous areas with hyperkeratotic plaques, accompanied by pruritus. Histologically, it is characterized by large, pale cells with mucicarmine-positive cytoplasm, isolated or in aggregates, in the epidermis. Case A 75-year-old woman presented with erythematous plaques covering the entire vulvar, vaginal and perianal area and pruritus. Smears were taken from all 3 sites and stained with Papanicolaou stain. Additionally, biopsies were taken from the vulva, vagina and outer borders of the lesion and were stained with hematoxylin-eosin and also for periodic acid Schiff stain, CK7, CEA, S-100 and CK20. The cytologic examination revealed numerous round to columnar, moderately enlarged atypical cells, dispersed or in loose groups, with abundant clear cytoplasm, vesicular nuclei and prominent nucleoli. The histologic findings confirmed the cytologic diagnosis. No indication of an underlying adenocarcinoma was found. Conclusion Although the histologic examination of a vulvar lesion is necessary in order to confirm the diagnosis of vulvar Paget's disease and rule out the possibility of an underlying invasive adenocarcinoma, the cytologic examination of vulvar smears is useful for alerting the clinician to the possibility of vulvar Paget's disease. (Acta Cytol 2010;54:898-902)