<prompt> Extra mammary Pagets involvesVulvaVaginaCervixUterus  <response>: A Vulva?Pagets Disease of the Vulva:Also known as extra mammary Pagets Disease.Represents close to 1 of all vulvar cancers.Occurs in the late reproductive post menopausal age group.Presents as pruritic burning, red, crusted patches interspersed with white ulcerated areas.Can be very extensive multifocal.95 of cases it is confined to the epidermis, considered a primary cutaneous in-situ adenocarcinoma arising from either pluripotential cells or from sweat glands.5 of cases it represents secondary vulvar skin involvement by a regional cancer.Microscopically it is characterized by an intraepithelial proliferation of malignant cells with vesicular nuclei cytoplasmic mucin.In about 30 of cases it can invade the dermis.It is very difficult to excise tends to recur.If it invades the dermis for a distance of more than 1mm it has metastatic potential.Pagets Disease:A rare extra mammary disease.Apocrine sweat glands are involved.Slighted elevated, sharply demarcated, white induratedeczematous lesion found in post-menopausal woman do pruritis.Biopsy reveals typical large pale vacuolated cells in epidermis. These Pagets cells are adeno carcinomatous mucus secreting round cells with vesicular nucleus and pale cytoplasm.Treatment - Vulvar Pagets Disease:Local excision or vulvectomy depend upon the extent of disease.Poor prognostic markers: Greater depth of invasion lymphovascular involvement.Mohs micrographic surgery: Lower recurrence rate.RT or CT?Long-term FU high risk of recurrenceAnnually inspection of vulva survey tumors at other site breast, lung, colorectum, gastric, pancreas, ovary?Extramammary Pagets Disease:This is rare lesion of the vulva and some times the perianal region presents as a pruritic, red, sharply demarcated, area.Most commonly affects the Labia majora:-In contrast to Pagets disease of the nipple, in which 100 of patients show an underlying ductal breast carcinoma, vulvar lesions are most frequently confined to the epidermis of the skin.?Extramammary Pagets Disease EMPD:Like that of breast, is essentially a form of intraepithelial carcinoma.Unlike breast, vulvar Pagets disease have no demonstrable underlying carcinoma.Microscopically, red, scaly plaque; characterized by spread of malignant cells within the epithelium, occasionally with invasion of underlying dermis.It is a rare, slow-growing, usually noninvasive intra-epithelial in the skin adenocarcinoma outside the mammary gland and includes Pagets disease of the vulva and the extremely rare Pagets disease of the penis. 