Abstract
Extramammary Paget disease is a rare neoplastic condition that more commonly affects postmenopausal Caucasian women. Although the vulvar area is the most frequently affected location, it corresponds solely to 1 to 2% of all vulvar malignancies. A 72-year-old female patient was observed in our outpatient clinic with a 2-year history of an erythematous and pruritic plaque on the vulva. Histopathology and immunohistochemistry studies were compatible with extramammary Paget disease of the vulva. Associated neoplastic conditions were excluded. Due to multiple relapses, the patient was submitted to three surgical interventions, including a total vulvectomy, and to external radiotherapy. The present case illustrates the chronic and recurrent nature of extramammary Paget disease despite aggressive procedures as well as the challenge in obtaining tumor-free resection margins.
Highlights
Extramammary Paget disease (PD) is a rare entity,[1,2,3,4] accounting for only 6% of all PD
Paget disease of the vulva (PDV) is a rare malignancy that should be considered in the differential diagnosis of welldefined, erythematous and scaly plaques in the vulvar region, in Caucasian postmenopausal women.[2,3,5,7]
While some studies suggest an intraepidermal origin in adnexal structures, recent theories have postulated a possible origin in Toker cells or in the mammary glands located in the interlabial sulcus.[7]
Summary
Rita Bouceiro-Mendes[1] Maria Mendonça-Sanches[1] Luís Soares-de-Almeida[1,2] Isabel Correia-Fonseca[1].
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Schedule a callMore From: Revista Brasileira de Ginecologia e Obstetrícia / RBGO Gynecology and Obstetrics
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