BACKGROUND. An effective chemotherapy for advanced extramammary Paget's disease has not yet been established. Recently, a low dose 5-fluorouracil/cisplatin (FP) regimen has been reported to be effective for adenocarcinoma, including gastric and colon carcinoma. However, this chemotherapeutic option has not been evaluated as to its effectiveness for extramammary Paget's disease. OBJECTIVE. We evaluated chemotherapy in a patient with advanced extramammary Paget's disease of the male genitalia unassociated with an underlying malignancy. METHODS. In order to treat a patient with extramammary Paget's disease who had multiple metastases, 500 mg/day of 5-fluorouracil (7 days per week) and 5 mg/day of cisplatin (5 days per week) were administrated intravenously for 24 hours and 1 hour, respectively. This protocol was continued for 6 weeks. RESULTS. A partial response was observed in both primary disease and metastatic disease. The primary tumor resolved almost entirely, leaving only a scar. Computed tomography scan revealed liver metastases that appeared to change into necrotic tissue; the metastases in lymph nodes and adrenal glands were markedly decreased and hardly detectable. In addition, the carcinoembryonic antigen level, a useful tumor marker for extramammary Paget's disease, decreased remarkably. It is suggested that this patient's survival period could have been prolonged. Serious side effects were not observed during this treatment. CONCLUSION. In one patient with extramammary Paget's disease and multiple metastases, low-dose FP therapy appeared to be beneficial. This regimen may be effective for extramammary Paget's disease with systemic nodular metastasis as an adjuvant therapy combined with surgery.
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