Extra mammary Paget's involves
(A) Vulva[?]Paget's Disease of the Vulva:Also known as extra mammary Paget's 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.Paget's Disease:A rare extra mammary disease.Apocrine sweat glands are involved.Slighted elevated, sharply demarcated, white indurated/eczematous lesion found in post-menopausal woman do pruritis.Biopsy reveals typical large pale vacuolated cells in epidermis. These Paget's cells are adeno carcinomatous mucus secreting round cells with vesicular nucleus and pale cytoplasm.Treatment - Vulvar Paget's Disease:Local excision or vulvectomy depend upon the extent of disease.Poor prognostic markers: Greater depth of invasion & lymphovascular involvement.Moh's micrographic surgery: Lower recurrence rate.RT or C/T?Long-term F/U (high risk of recurrence)Annually inspection of vulva & survey tumors at other site (breast, lung, colorectum, gastric, pancreas, ovary)[?]Extramammary Paget's 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 Paget's 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 Paget's Disease (EMPD):Like that of breast, is essentially a form of intraepithelial carcinoma.Unlike breast, vulvar Paget's 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 Paget's disease of the vulva and the extremely rare Paget's disease of the penis.
Radiotherapy
Vulva
Vagina
Cervix
Uterus
Vulva