Combination chemotherapy for metastatic extramammary Paget disease

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It is difficult to treat patients in the advanced stages of extramammary Paget disease (EMPD) because no effective treatment has yet been established.


To describe the experience of using combination chemotherapy (FECOM) in patients with metastatic EMPD.


Since we reported a case of metastatic EMPD that responded to FECOM, we have treated further patients with metastatic EMPD using FECOM at the National Cancer Center Hospital in Japan. FECOM consists of epirubicin 40 mg m−2, mitomycin C 3·5 mg m−2 and vincristine 0·7 mg m−2 on day 1, carboplatin 300 mg m−2 on day 2 and 5-fluorouracil 350 mg m−2 on days 2–6. To evaluate the efficacy of this combination therapy in patients with metastatic EMPD, data regarding patients given FECOM for the first-line treatment of metastatic EMPD were extracted retrospectively.


Seven patients were eligible for this study. A partial response was noted in four evaluable patients (100%). The other three patients were not evaluable for clinical response. One of the three unevaluable patients showed a decrease in tumour size by 100%, the other two by about 20%. The median overall survival and progression-free survival were 9·4 months (7·6–17·3) and 6·5 months (2·6–7·9), respectively. The 1-year survival rate was 43% (three of seven). Three of the seven patients (43%) had grade 3 haematological toxicities. All treatment-related toxicities were reversible and there was no febrile neutropenia or treatment-related deaths.


This study suggests that the combination chemotherapy FECOM may be a treatment option for patients with metastatic EMPD.


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