Combination chemotherapy for metastatic extramammary Paget disease

Summary Background It is difficult to treat patients in the advanced stages of extramammary Paget disease ( EMPD ) because no effective treatment has yet been established. Objective To describe the experience of using combination chemotherapy ( FECOM ) in patients with metastatic EMPD . Methods 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. Results 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. Conclusion This study suggests that the combination chemotherapy FECOM may be a treatment option for patients with metastatic EMPD .