Extramammary Paget Disease:a Case Report And Review Of The Literature

OBJECTIVE:To review the experience on diagnosis and treatment for extramammary Paget disease.METHOD(S):Reported a case of extramammary Paget disease including the syndrome, laboratory results, pathological examination results, the diagnosis and treatment followed by a review of the current literature.DESIGN:A case report.PATIENT:A65-year-old male has presented with a red plaque on his right groin and scrotum with mild pruritus for2years and has been more severe for one month. With erosion and exudate, the erythematous lesion had a relatively clear boundary, about8cm×5cm, which was covered by some crust. Histopathological examination revealed hyperkeratinization, stratum spinosum hyperplasia, tumor cells forming small strands and nests in epidermis and dermis, breaking through the basement membrane. Part of tumor cells were large with abundant eosinophilic cytoplasm and vacuolated nuclei, in which there were several obvious nucleoli. Immunohistochemical stain showed positive staining for CKH, CK7, CEA, EMA and weakly positive staining for c-erbB-2.INTERVENTION(S):Application of surgical treatment after biopsy and histopathological examination results.RESULT(S):With the clinical presentation and relative examination, the patient was diagnosed as extramammary Paget disease. No internal tumor was found. The patient had a wide lesion excision and right inguinal lymph nodes dissection. No lymph nodes metastasis was found, He discharged after getting better, and1year later the follow-up showed no relapse.CONCLUSION(S):Extramammary Paget disease is a rare epidermal malignant tumor that presents mainly on apocrine gland-bearing skin, often accompanied by various kinds of internal malignancies. The tumor is the malignancy of apocrine gland and mammary duct cells. It usually occurs in senior males, and presents as an unilateral red or brown plaque on apocrine gland-bearing skin. Its diagnosis should be confirmed with histopathological examination and immunohistochemical staining, and positivity of CK-7is a sensitive signal. General physical examination is suggested to assure whether internal tumors accompany or not. The main treatment is wide lesion excision in our country, but recurrence rate is high despite aggressive surgeries. So some alternative modalities are being explored. Judging the prognosis largely depends on there is dermis and lymph nodes metastasis or not.