OBJECTIVETo evaluate the clinical manifestation, management and prognostic characteristics of scrotal extramammary Pagets disease (EMPD).

METHODSTwenty-three cases of EMPD were identified and retrospectively reviewed, and the clinical findings, surgical treatment, pathologic features and prognostic characteristics were evaluated. The patients ranged in age from 49 to 72 years (mean 62. 4 years). The diagnoses were delayed by 12 to 132 months (67. 6 on average) in 9 cases at Stage A1 , 12 at A2, 1 at B and 1 at D. The rate of initial misdiagnosis was 91.3% (21/23). Twenty of the patients underwent operations, 2 received radiotherapy and 1 chemotherapy.

RESULTSTwenty-two patients of the total number were followed up for 7 to 223 months, averaging at 119, therapy and 1 chemotherapy. of whom 12 remained cancer free, 1 died of tumor, 3 died of intercurrent disease, 4 experienced local recurrence, 1 relapsed with inguinal lymph node metastasis and 1 developed inguinal lymph node metastasis exclusively. Those with relapses received the second surgery, and 5 of them survived without cancer and 1 died of intercurrent disease.

CONCLUSIONThe primary treatment of choice for scrotal EMPD is radical excision. Precise, histological examination before and during the operation is of crucial importance. The positive excisal margins are one of the key causes of local recurrence, and the involvement of dermis by Pagets cells may suggest possible metastasis. Scrotal EMPD tends to occur as a slowly growing lesion, mainly in the elderly, and has a good prognosis in cases of non-invasion. Otherwise the prognosis is poor.