RESUMO
PURPOSE: Extramammary Paget's disease (EMPD) is a rare neoplasm with only a limited number of cases reported in the literature. Little was known about the availability of systemic chemotherapy for metastatic EMPD. METHODS: We report one case of heavily pretreated EMPD with multiple organ metastases and successfully treated by pemetrexed. RESULT: The tumor was progression after multi-line therapy including erlotinib, radiotherapy, combined chemotherapy and radioactive particles implantation. Pemetrexed monotherapy was applied and progression free survival of more than 5 months with partial remission (PR) response was achieved. Only 1 time of grade 3 neutropenia was observed during the pemetrexed chemotherapy. CONCLUSION: Due to the significant response and tolerability in the present case, pemetrexed monotherapy was recommended as a potent candidate for patients with advanced EMPD.