title: Combination chemotherapy for metastatic extramammary Paget disease.
abstract: 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.
  
  
  CONCLUSIONS
  This study suggests that the combination chemotherapy FECOM may be a treatment option for patients with metastatic EMPD.
authors: Department of Dermatologic Oncology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045, Japan; Department of Plastic and Reconstructive Surgery, Graduate School of Medicine, University of Hokkaido at Sapporo, Kita-15 Nishi-7, Kita-Ku, Sapporo Hokkaido, 060-8638, Japan.
authors: K
authors: K
authors: Oashi
authors: A
authors: A
authors: Tsutsumida
authors: K
authors: K
authors: Namikawa
authors: R
authors: R
authors: Tanaka
authors: W
authors: W
authors: Omata
authors: Y
authors: Y
authors: Yamamoto
authors: N
authors: N
authors: Yamazaki
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001005:Anus Neoplasms / Q000188:drug therapy* / Q000601:surgery; D001859:Bone Neoplasms / Q000556:secondary; D016190:Carboplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D015251:Epirubicin / Q000008:administration & dosage; D005260:Female; D005472:Fluorouracil / Q000008:administration & dosage; D005834:Genital Neoplasms, Male / Q000188:drug therapy* / Q000601:surgery; D006801:Humans; D008113:Liver Neoplasms / Q000556:secondary; D008197:Lymph Node Excision; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D016685:Mitomycin / Q000008:administration & dosage; D010145:Paget Disease, Extramammary / Q000188:drug therapy* / Q000601:surgery; D012189:Retrospective Studies; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage; D014846:Vulvar Neoplasms / Q000188:drug therapy* / Q000601:surgery
chemical_list: D015251:Epirubicin; D016685:Mitomycin; D014750:Vincristine; D016190:Carboplatin; D005472:Fluorouracil