Pemetrexed induced a durable response in heavily pretreated metastatic extramammary Paget's disease.

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.