title: Clinical outcomes of adult and childhood rhabdomyosarcoma treated with vincristine, d-actinomycin, and cyclophosphamide chemotherapy.
abstract: BACKGROUND
  Outcomes in adult patients with rhabdomyosarcoma are poor, with a 5-year survival rate of approximately 30 %. The current study aimed to compare the clinical outcomes of adult and childhood rhabdomyosarcoma patients with local and metastatic disease and to examine the impact and timing of local therapy on metastasis.
  
  
  METHODS
  Clinicopathological features and patient outcomes were reviewed retrospectively for rhabdomyosarcoma patients receiving chemotherapy between 1981 and 2010 at our institution. Adults were defined as those aged 21 years or older.
  
  
  RESULTS
  Of the 98 patients identified, 36 were adults (median age, 29; range, 21-72) and 62 were children (median age, 11; range, 0.6-20). Median progression-free survival of localized and metastatic disease for children and adults was as follows: localized disease, 166.9 versus 22.4 months (p = 0.005), and metastatic disease, 13.3 versus 13.3 months (p = 0.949), respectively. Multivariate regression analysis revealed that older age (≥ 21 vs. <21) was a significant poor prognostic factor in localized disease. Conversely, age was not related to survival in metastatic disease. Receiving radiotherapy to the primary site was an independent factor indicating a better prognosis. An analysis of the optimal timing of local therapy was performed for 53 patients; however, its significance on survival could not be determined.
  
  
  CONCLUSIONS
  Age was a negative prognostic factor in rhabdomyosarcoma patients with localized disease, but it did not affect the survival in metastatic disease. For metastatic disease, although local therapies may be effective for survival, the timing of such therapies should be determined individually.
authors.affiliation: Department of Medical Oncology and Breast Oncology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045, Japan.
authors.forename: Yuki
authors.initials: Y
authors.lastname: Kojima
authors.forename: Kenji
authors.initials: K
authors.lastname: Hashimoto
authors.forename: Masashi
authors.initials: M
authors.lastname: Ando
authors.forename: Kan
authors.initials: K
authors.lastname: Yonemori
authors.forename: Akihiro
authors.initials: A
authors.lastname: Hirakawa
authors.forename: Makoto
authors.initials: M
authors.lastname: Kodaira
authors.forename: Mayu
authors.initials: M
authors.lastname: Yunokawa
authors.forename: Chikako
authors.initials: C
authors.lastname: Shimizu
authors.forename: Kenji
authors.initials: K
authors.lastname: Tamura
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Katsumata
authors.forename: Ako
authors.initials: A
authors.lastname: Hosono
authors.forename: Atsushi
authors.initials: A
authors.lastname: Makimoto
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Fujiwara
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000188:drug therapy* / Q000473:pathology; D015996:Survival Rate; D014750:Vincristine / Q000008:administration & dosage; D055815:Young Adult
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D003520:Cyclophosphamide