Most common site for Extra-mammary Paget&;s Disease is:
Vulva is the most common site of involvement in Extramammary Paget&;s disease. Extra-Mammary Paget Disease (EMPD): Extramammary Paget&;s disease is a rare intraepithelial malignancy involving the anogenital region or axilla (less commonly) The most common site of involvement in Extramammary Paget&;s disease is the vulva. Extramammary Paget&;s disease can be either a primary cutaneous adenocarcinoma or a tumor with secondary cutaneous involvement extension from a lower gastrointestinal or urinary tract carcinoma. Mammary Paget&;s disease is viually always associated with an underlying ductal carcinoma of the breast. This constant association with an underlying carcinoma doesnot peain in Extramammary Paget&;s disease. The true cell of origin for primary cutaneous Extra-mammary paget disease is controversial. Mostly cases are believed to arise from underlying apocrine gland. Extra-mammary paget disease may also arise from pleuripotent stem cells or Toker cells. The usual clinical presentation of Extramammary Paget&;s disease, irrespective of site, is an erythematous patch or plaque that may be scaly, eroded, or sometimes ulcerated. Pruritus is the most common symptom. Multifocal and bilateral tumors have been repoed. Histopathologically, Extra-mammary Paget disease mimic&;s Paget disease of the breast. Ref: Pathology of Incipient Neoplasm (Oxford University Press) 3rd edition Pgno: 227
Uterus
Vulva
Vagina
Ovary
Breast