Aggressive surgery is unwarranted for biliary tract rhabdomyosarcoma

Sheri L. Spunt, Thom E. Lobe, Alberto S. Pappo, David M. Parham, Moody D. Wharam, Carola Arndt, James R. Anderson, William M. Crist, Charles Paidas, Eugene Wiener, Richard J. Andrassy, Cindy L. Schwartz

Research output: Contribution to journalArticlepeer-review

90 Scopus citations


Background/Purpose: 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.

Original languageEnglish (US)
Pages (from-to)309-316
Number of pages8
JournalJournal of pediatric surgery
Issue number2
StatePublished - Feb 2000


  • Bile duct neoplasms
  • Biliary tract neoplasms
  • Biliary tract surgeries
  • Intergroup Rhabdomyosarcoma Study Group
  • Rhabdomyosarcoma
  • Surgery

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health


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