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Vulvar Extramammary Paget's Disease: Clinicopathologic Features and Oncologic Outcomes

, Francesc Fargas, Francesc Tresserra, Ariel Glickman, Sonia Baulies, Pere Barri-Soldevila, Rafael Fábregas
Published: 12 December 2022

Abstract:Objective: Vulvar extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma of the vulvar skin. This study evaluated the clinical characteristics, treatment modalities, and oncologic outcomes in patients with vulvar EMPD. Materials and Methods: Cases of patients who were treated for vulvar EMPD between 1994 and 2022 were identified. Patient demographics, associated malignancies, treatments, histopathologic features, need for re-operations, and long-term outcomes were documented. Results: The study involved 10 patients with vulvar noninvasive EMPD, with a median age of 57 (31–86) years. None of the patients had prior malignancies. The index operations performed were wide local excision with reconstructive surgery (5 patients) and vulvectomy with reconstructive surgery (3 patients). Seven patients were positive for margin involvement. Five patients developed recurrences. The mean time of the first recurrence was 32.6 (4–88) months. All recurrences were treated with reoperations. Only 1 associated malignancy was found: cervical cancer. There was no disease progression in any of the patients who were followed; of the 10 patients, 2 were lost to follow-up. Conclusions: EMPD is characterized by a high tendency to recur after surgical resection. Surgical management can be a challenge due to the high rate of residual tumors in the surgical margins. (J GYNECOL SURG 20XX:000)
Keywords: extramammary Paget's disease / intraepithelial adenocarcinoma / vulvar cancer / surgical management

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