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Revista Brasileira de Ginecologia e Obstetrícia. 2004;26(4):329-335
DOI 10.1590/S0100-72032004000400011
Extramammary Paget's disease (EPD) is an uncommon neoplasic condition observed mostly in areas with numerous apocrine and or eccrine glands. In the woman it is most commonly seen on the vulva, although it can occur in other locations. Vulvar Paget's disease (VPD) can be classified into primary, of cutaneous origin, and secondary, of extracutaneous origin, with significant clinical e prognostic implications. Clinically VPD begins insidiously with pruritus and burning sensation. The lesion appears as a solitary patch with an eczematous, erythematous and squamous surface. This is a report of a case of a 72-year-old patient with an erythematous slightly thickened patch lesion with spots of erosion involving both the right and the left majus and minus labia, the clitoris, the pubic region, and the perineal and perianal regions. The operation performed included radical vulvectomy and bilateral inguinal lymphadenectomy. The histopathology revealed invasive Paget's disease. Immunohistochemical methods showed positive Paget cells for CEA, EMA, and cytokeratin pan. Pathogenesis and diagnosis of EPD is discussed, with differential diagnosis and reference to immunohistochemical methods. Recurrence rate is 30%, even with margin control. Experience with EPD is limited and long-term follow-up is required to exclude recurrence of the disease and development of an associated cancer.
Summary
Revista Brasileira de Ginecologia e Obstetrícia. 2004;26(4):329-335
DOI 10.1590/S0100-72032004000400011
Extramammary Paget's disease (EPD) is an uncommon neoplasic condition observed mostly in areas with numerous apocrine and or eccrine glands. In the woman it is most commonly seen on the vulva, although it can occur in other locations. Vulvar Paget's disease (VPD) can be classified into primary, of cutaneous origin, and secondary, of extracutaneous origin, with significant clinical e prognostic implications. Clinically VPD begins insidiously with pruritus and burning sensation. The lesion appears as a solitary patch with an eczematous, erythematous and squamous surface. This is a report of a case of a 72-year-old patient with an erythematous slightly thickened patch lesion with spots of erosion involving both the right and the left majus and minus labia, the clitoris, the pubic region, and the perineal and perianal regions. The operation performed included radical vulvectomy and bilateral inguinal lymphadenectomy. The histopathology revealed invasive Paget's disease. Immunohistochemical methods showed positive Paget cells for CEA, EMA, and cytokeratin pan. Pathogenesis and diagnosis of EPD is discussed, with differential diagnosis and reference to immunohistochemical methods. Recurrence rate is 30%, even with margin control. Experience with EPD is limited and long-term follow-up is required to exclude recurrence of the disease and development of an associated cancer.
Summary
Revista Brasileira de Ginecologia e Obstetrícia. 2003;25(4):283-288
DOI 10.1590/S0100-72032003000400010
High-grade vulvar intraepithelial neoplasia (VIN III) is a visible lesion; therefore, it is accessible to biopsy and thus, to a histological diagnosis. There are two forms of vulvar cancer precursors: VIN caused by human papillomavirus (HPV) and VIN associated with untreated lichen simplex chronicus, squamous cell hyperplasia, and lichen sclerosus. There may be overlap of the two forms. The term bowenoid papulosis, although discouraged, identifics a clinical form of VIN III. Such lesion appears as pigmented, wart-like growths or papules. VIN III is associated with HPV in more than 80% of the cases, and there is perianal involvement in 40% of the times. Vulvar intraepithelial neoplasia is difficult to cure and relapses can occur at any time for many years. Although there is no defined standard treatment, studies point to surgery, respecting a free margin, as the most adequate one.
Summary
Revista Brasileira de Ginecologia e Obstetrícia. 2003;25(4):283-288
DOI 10.1590/S0100-72032003000400010
High-grade vulvar intraepithelial neoplasia (VIN III) is a visible lesion; therefore, it is accessible to biopsy and thus, to a histological diagnosis. There are two forms of vulvar cancer precursors: VIN caused by human papillomavirus (HPV) and VIN associated with untreated lichen simplex chronicus, squamous cell hyperplasia, and lichen sclerosus. There may be overlap of the two forms. The term bowenoid papulosis, although discouraged, identifics a clinical form of VIN III. Such lesion appears as pigmented, wart-like growths or papules. VIN III is associated with HPV in more than 80% of the cases, and there is perianal involvement in 40% of the times. Vulvar intraepithelial neoplasia is difficult to cure and relapses can occur at any time for many years. Although there is no defined standard treatment, studies point to surgery, respecting a free margin, as the most adequate one.
