Extramammary Paget disease of the vulva: Management and prognosis

Objective: To evaluate the clinicopathological characteristics, management and prognosis of patients with vulvar extramammary Paget disease of the vulva (EMPD).

Materials and methods: The U.S National Cancer Database was accessed and patients diagnosed between 2004 and 2015 with microscopically confirmed vulvar EMPD were selected. Overall survival (OS) was calculated for patients diagnosed between 2004 and 2014, who had at least one month of follow-up. Five year OS rates were calculated following generation of Kaplan-Meier curves while comparisons were made with the log-rank test.

Results: A total of 2602 patients were identified. Median age at diagnosis was 72 years (range 31-90 years) and the majority were of White race (92%), without any co-morbidities (80.9%). Personal history of another tumor was present in 36.9% of patients. In situ EMPD was diagnosed in 994 cases (38.2%) and the majority (95.1%) were managed with local excision or vulvectomy. Five-year OS was 85.8%, while presence of positive margins was not associated with worse OS (p = 0.38). Invasive EMPD was diagnosed in 1608 (61.8%) patients. Staging information was available for 1172 patients, 75.3% had early stage disease. Most patients underwent surgical treatment (91.6%); 53.6% had positive margins. Performance of lymphadenectomy was infrequent (6.8%). Moreover, immunotherapy (4.5%), chemotherapy (1.5%) and radiation therapy (2.2%) were rarely employed in the management of invasive EMPD. Patients with early stage disease (n = 766) had better OS compared to those with advanced stage (n = 278) (5-yr OS rates were 84.3% and 73.6% respectively, p = 0.015) while presence of positive margins was not associated with worse OS (p = 0.35).

Conclusions: Extramammary Paget disease is a rare vulvar tumor. Surgical excision is the main treatment option while other modalities are rarely employed. Overall survival rates are encouraging.