Extramammary paget disease is characterized by the consistent lack of estrogen and progesterone receptors but frequently expresses androgen receptor

Extramammary Paget disease (EPD) is art uncommon cutaneous malignant neoplasm that arises in areas rich in apocrine glands (perineum, vulva, and axilla). Apocrine gland origin or apocrine differentiation of cells of EPD has been suggested. Estrogen, progesterone, and androgen hormone receptors have been reported to exhibit a characteristic pattern of expression in mammary apocrine type carcinomas; however their expression in EPD has not been elucidated fully. By using immunohistochemical methods, we studied the expression of steroid receptors in EPD on formalin-fixed paraffin-embedded tissue samples from 28 patients with EPD tr without associated visceral malignant neoplasms or adnexal carcinoma. Androgen receptor (AR) was identified in 15 of 28 cases. The proportion of AR-positive cells varied from 1% to more than 75%; 8 cases expressed AR in more than 10% of cells. Strong AR expression also Mns seen in the invasive carcinoma arising from 1 case of EPD. All cases lacked immunohistochemically, detectable estrogen and progesterone receptors. The immunophenotype characteristic of apocrine carcinomas (AR-positive, estrogen receptor-negative, progesterone receptor-negative) was seen in a substantial proportion of EPD cases. Results suggest that AR expression is a factor in pathogenesis of EPD. This may? be important for the therapy of recurrent or invasive disease. Extramammary Paget disease (EPD) is an intraepidermal adenocarcinoma that exhibits features of apocrine differentiation. It is typified by an erythematous eczematoid appearance of the skin mostly in the areas rich in apocrine glands (genitoperineal region and axillae). The disease tends to occur in middle-aged or elderly patients and more frequently in women.(1.2) This tumor nevertheless possesses the potential for dermal invasion and distant metastasis.(3-5) Although surgical treatment remains the primary treatment modality for EPD, the recurrence rate approaches 50%.(6) For such cases, topical chemotherapy with fluorouracil(7) and radiotherapy(8) have been used with variable efficacy. Normal apocrine glands of the skin(9) and mammary apocrine lesions (metaplasias and carcinomas) contain no detectable estrogen receptors (ERs) or progesterone receptors (PRs), but consistently express androgen receptors (ARs).(10) These findings prompted us to study the expression of steroid hormone receptors in EPD. In selected cases, androgen blockage may have therapeutic potential in advanced disease.