Vulvectomy

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Navigating the Maze: Insights from a Case Series of Vulval Extramammary Paget’s Disease

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Navigating the Maze: Insights from a Case Series of Vulval Extramammary Paget’s Disease

Extramammary Paget’s remains a rare neoplastic condition, which although shares the name with Paget’s disease of the breast, is a distinct entity. The condition predominantly affects the epidermis of the vulval skin of postmenopausal women. In this case series, we present the cases of two geriatric patients who were diagnosed with this rare condition and the different treatment options available in managing the disease. Despite its rarity, understanding of this neoplastic condition is vital due to its potential for misdiagnosis and its impact on patient quality of life.

A Case of Chronic and Relapsing Paget Disease of the Vulva

A Case of Chronic and Relapsing Paget Disease of the Vulva

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.

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