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.
Vulva
Vagina
Cervix
Uterus
Radiotherapy