Case Report 303Urology Journal Vol 6 No 4 Autumn 2009 Extramammary Paget’s Disease in Prostate Amit Kumar Yadav, Richa gupta, Usha Rani Singh Urol J. 2009;6:303-5. www.uj.unrc.ir Keywords: extramammary Paget’s disease, prostatic neoplasms, tissue polypeptide antigen Department of Pathology, University College of Medical Sciences, New Delhi, India Corresponding Author: Amit Kumar Yadav, MD Department of Pathology, University College of Medical Sciences, Dilshad Garden, New Delhi – 110095, India Tel: +91 11 2258 6262 E-mail: amityadav7284@yahoo.co.in Received October 2008 Accepted February 2009 INTRODUCTION Extramammary Paget’s disease is a condition morphologically similar to Paget’s disease in the breast, but occurs outside the breast. It is an uncommon condition as compared to its mammary counterpart. It most commonly affects the vulva, less commonly the male genital area or the perianal area.(1,2) To the best of our knowledge, extramammary Paget’s disease has not been described in prostate previously. We therefore report this particular case. CASE REPORT A 70-year-old male presented to the surgery outpatient department with complaints of dribbling micturition and nocturia since 2 years earlier. There was also a history of episodes of urinary retention which had been relieved by catheterization. The patient had received medical therapy on 2 occasions; however, it failed to bring any relief to the patient. Transrectal ultrasonography showed an enlarged prostate weighed 37 g. Serum prostate- specific antigen level was 3.4 ng/ mL. Based on the above clinical presentation, a decision to carry out transurethral resection of the prostate was made. Multiple prostatic chips together measuring 6 × 4 × 2 cm were received in the histopathology department. Microscopic examination predominantly showed features of nodular hyperplasia of the prostate (Figure 1), with a single bit of tissue lined by stratified squamous epithelium, showing Paget’s cells within the epidermis (Figures 2 and 3). These cells were found to be more in number in the lower layers Figure 2. Single tissue bit lined by stratified squamous epithelium showing Paget’s cells in the lower layers of epidermis (× 100). Figure 1. Morphological features of nodular hyperplasia prostate (× 100). Paget’s Disease in Prostate—Yadav et al 304 Urology Journal Vol 6 No 4 Autumn 2009 of the epidermis as compared to the upper layers. The Paget’s cells were present as single isolated cells or in small groups. They were enlarged and had large nuclei with pale cytoplasm. In view of the above morphological features, a diagnosis of extramammary Paget’s disease was made. We considered the possibility of invasion to the urinary bladder, prostate, external genitalia, and anal canal. Cystoscopic examination of the bladder did not reveal any visible growth in the bladder. The prostatic tissue submitted in the specimen also did not demonstrate any evidence of malignancy. Physical examination of the external genitalia and the anal canal did not reveal any visible growth. Immunohistochemistry was then performed on the tissue sections using cytokeratin 7 (CK7) and cytokeratin 20 (CK20) antibodies. The Paget’s cells were weakly positive for CK7, interpreted as negative (Figure 4) and negative for CK20 (Figure 5). Based on the above immunohistochemistry findings, a primary prostatic origin of extramammary Paget’s disease was suggested. A thorough physical examination and detailed investigations carried out did not reveal any associated pathologic findings. DISCUSSION Paget’s disease most commonly affects the vulva, and less commonly, the male genital area or the perianal area. In exceptional cases only, the axilla, the region of the ceruminous glands, or that of Moll’s glands are reported to be affected by extramammary Paget’s disease.(3,4) In cases with the involvement of the axilla, the genital area may also be affected. Thus, extramammary Paget’s disease involves areas in which apocrine glands are normally encountered. In the rare instances, extramammary Paget’s disease is a secondary event caused by extension of an adenocarcinoma either of the rectum to the perianal region, of the cervix to the vulvar region, or of the urinary bladder to the urethra and glans penis, or to the groin.(2,5-8) Extramammary Paget’s disease has not been reported previously in the prostate (transurethral resection of the prostate chips). However, various authors have reported cases of carcinoma originating in ducts surrounding the prostatic urethra and in intraductal carcinoma. (9,10) Ullman and Ross(11) described features of hyperplasia, atypia, and carcinoma in situ in prostatic periurethral glands. Transitional cell carcinoma has also been reported in the prostate, previously. (12-15) Immunohistochemical findings of Figure 4. Paget’s cells showing weak positivity for cytokeratin 7 (× 400). Figure 3. Paget’s cells in higher magnification (× 400). Figure 5. Paget’s cells negative with cytokeratin 20 (× 400). Paget’s Disease in Prostate—Yadav et al Urology Journal Vol 6 No 4 Autumn 2009 305 CK7- /CK20- helped us suggest a diagnosis in this case. Lopez-Beltran and coworkers(16) suggested that a panel of immunostains including CK7/ CK20 might assist in differentiating urothelial carcinoma (CK7+/CK20+) from extramammary Paget’s disease of anorectal origin which is known to be CK7+/CK20-. As the present case was negative for both CK7 and CK20, possibility of a prostatic origin was suggested. 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