IMR Press / EJGO / Volume 20 / Issue 2 / pii/1999125

European Journal of Gynaecological Oncology (EJGO) is published by IMR Press from Volume 40 Issue 1 (2019). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on as a courtesy and upon agreement with S.O.G.

Case Report

Extramammary Paget's disease of the vulva: report of five cases and review of the literature

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1 Unit of Gynecologic Oncology; Department of Obstetrics and Gynecology, Soroka Medical Center and Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel
2 Department of Obstetrics and Gynecology, Kaplan Medical Center, Rehovot, Israel
Eur. J. Gynaecol. Oncol. 1999, 20(2), 98–101;
Published: 10 April 1999

Five patients with vulvar extramammary Paget's disease (EMPD) are reported. They accounted for 4.4% of all vulvar malignan­c1es diagnosed in the south of Israel between 1961 - 1997. Mean age was 64.4 years and the predominant symptom was vu Ivar pruritus. Three patients had intra-epidermal lesions (managed by wide local excision or, at most, simple vulvectomy), one had a mini­mally invasive lesion (managed by simple vulvectomy) and one had EMPD with an underlying apocrine gland adenocarcinoma (managed by radical vulvectomy and bilateral groin dissection followed by pelvic radiotherapy). EMPD recurred in two patients 1) local recurrence after simple hemivulvectomy for an intra-epidermal lesion was successfully treated by wide local excision; 2) widespread recurrence at distant skin sites after simple vulvectomy for a minimally invasive lesion was unsuccessfully treated with systemic chemotherapy. It is concluded that vulvar EMPD is an uncommon neoplasm that primarily affects postmenopausal women Its histogenesis is uncertain and most commonly it is an intra-epidermal lesion. The high rate of recurrent disease remains a chal­lenge for optimal management.

Paget's disease
Wide local excision
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