title: Aggressive surgery is unwarranted for biliary tract rhabdomyosarcoma.
abstract: OBJECTIVE
  Rhabdomyosarcoma (RMS) of the biliary tract is rare, and, in addition to multiagent chemotherapy with or without radiotherapy (RT), some investigators recommend aggressive surgery. To assess the role of surgery, records of all 25 eligible patients with biliary RMS enrolled in IRSG studies I through IV from 1972 to 1998 were reviewed.
  
  
  METHODS
  Treatment included surgery with or without vincristine, dactinomycin, cyclophosphamide, doxorubicin, cisplatin, etoposide, ifosfamide, and with or without RT. Data evaluated included clinical presentation, treatment, complications, and outcome.
  
  
  RESULTS
  Diagnostic imaging identified the primary tumor but failed to identify regional metastases. Despite aggressive surgery, gross total resection at diagnosis was possible in only 6 cases, 2 of which had negative surgical margins. Although only 6 (29%) patients without distant metastases underwent gross total resection, estimated 5-year survival rate was 78% (95% CI 58%, 97%). Infectious complications were common and frequently associated with external biliary drains. Five (20%) died within the first 2 months, 3 of sepsis.
  
  
  CONCLUSIONS
  Surgery is critical for establishing an accurate diagnosis and determining the extent of regional disease. Gross total resection is rarely possible despite aggressive surgery, and outcome is good despite residual disease after surgery. External biliary drains increase the risk of postoperative infectious complications.
authors.affiliation: IRS Group of the Children's Cancer Group and the Pediatric Oncology Group, Rochester, MN 55905-0001, USA.
authors.forename: S L
authors.initials: SL
authors.lastname: Spunt
authors.forename: T E
authors.initials: TE
authors.lastname: Lobe
authors.forename: A S
authors.initials: AS
authors.lastname: Pappo
authors.forename: D M
authors.initials: DM
authors.lastname: Parham
authors.forename: M D
authors.initials: MD
authors.lastname: Wharam
authors.forename: C
authors.initials: C
authors.lastname: Arndt
authors.forename: J R
authors.initials: JR
authors.lastname: Anderson
authors.forename: W M
authors.initials: WM
authors.lastname: Crist
authors.forename: C
authors.initials: C
authors.lastname: Paidas
authors.forename: E
authors.initials: E
authors.lastname: Wiener
authors.forename: R J
authors.initials: RJ
authors.lastname: Andrassy
authors.forename: C L
authors.initials: CL
authors.lastname: Schwartz
mesh_terms: D001661:Biliary Tract Neoplasms / Q000401:mortality / Q000601:surgery* / Q000628:therapy; D001662:Biliary Tract Surgical Procedures; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012086:Reoperation; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000401:mortality / Q000601:surgery* / Q000628:therapy; D016896:Treatment Outcome