Administration of docetaxel for advanced extramammary Paget’s disease: a case report and retrospective study

Abstract

Extramammary Paget’s disease (EMPD) is an apocrine gland-derived skin neoplasm that is common in the elderly. In many cases, the prognosis is good after extended resection. However, the prognosis of advanced EMPD is poor as no effective chemotherapy has been established. Here we report a 73-year-old male patient with EMPD in the genital region who developed metastases in the inguinal area, pelvis, paraaortic lymph nodes (LNs), bone, and brain. Although treatment with docetaxel (DTX) was effective for the primary skin lesion and LN metastasis, the patient died of meningeal carcinomatosis (MC). In our department, DTX has been administered to seven cases of advanced EMPD, showing a partial response in three and progressive disease in four cases. Although DTX treatment was highly effective for LN metastases, complication of MC occurred in three fatal cases. It is expected that DTX will become the first-line chemotherapeutic regimen for advanced EMPD. Development of an additional treatment strategy for advanced EMPD with MC will be crucial.