Extramammary Paget's Disease of Vulva: A Clinicopathological Case Series of a Rare Disease

Purpose Extramammary Paget's disease (EMPD) is a rare cutaneous intraepithelial neoplasm of vulva. Due to the rarity of the tumor and limited literature, we intend to study the clinicopathological attributes of the tumor. Methods Consecutive cases of EMPD of vulva during 2009-2020 (12 years) were retrieved from the archives and a clinicopathological analysis was done. Results A total of 13 cases were retrieved and reviewed. The age range was 44-85 years (Mean: 61 years). All the patients presented with reddish to whitish patch over labia. The diagnosis on biopsy was rendered in 11 cases and on surgery in 2 cases, with the help of a panel of immunohistochemistry. The characteristic histopathological features of intraepithelial presence of clustered large cells with clear to pale cytoplasm with hyperchromatic nuclei were seen in all cases, with stromal invasion in one case. Surgery was performed in 8 cases (61.5%). Margin positivity on histopathological examination was noted in 3/6 (50%) cases, and margins were negative in 3/6 (50%) cases. Follow-up for at least 5 months was available for 9 cases (Median: 22.5 months; Mean: 25.9 months). Local disease recurrence was identified in 4/9 cases (44.4%), the duration of disease-free survival ranging from 18 to 68 months post-primary diagnosis. Two patients presented with progressive local disease (22.2%), wherein one patient was on treatment with EBRT and the other patient defaulted before any primary treatment. Conclusion EMPD is a disease with a chronic relapsing course. Accurate diagnosis on biopsy is fraught with challenges; however, is essential for optimal management. An underlying malignancy should always be ruled out.