Extramammary Paget's disease of the Vulva

Abstract

Vulvar Paget's disease is an extremely rare neoplasm that accounts for less than 1% of the vulvar malignancies. We present a case of a 75-year-old woman, who had an eczematoid lesion involving the labia majora and minora bilaterally, with infiltration to the clitoris. Enlarged non-fixed lymph nodes were palpable in the inguinal region bilaterally. A biopsy of the vulva showed Paget's disease. She underwent radical vulvectomy with bilateral inguinal lymph node dissection. The patient remained disease free at 6-month follow-up.

Extra Mammary Paget’s Disease of Vulva—a Case Report

“…She was planned for surgery at our center. She underwent wide excision of the lesion with a 2-cm gross margin, which amounted to en bloc removal of the bilateral labia majora, minora, and clitoris [2] (Fig. 1b).…”

Úlceras genitales en la mujer

“…La enfermedad de Paget extramamaria comienza clínicamente como una placa de aspecto eccematoso que crece en expansió n, pudiendo ulcerarse. Habrá que descartar su asociació n a neoplasias internas 73 . Otros carcinomas epiteliales raros en esta localizació n son el carcinoma basocelular y el carcinoma verrucoso.…”

Extramammary Paget Disease of the Vulva - Case Report

“…1,2 Vulvar Paget disease is more common from the 5th decade of life on, 2 with an average age at diagnosis from 61 to 69 years, depending on the study. [4][5][6][7][8] It is more common in Caucasians and is rare before menopause. 6,8 Extramammary Paget disease accounts for less than 1% of vulvar neoplasms.…”