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Aggressive surgery is unwarranted for biliary tract rhabdomyosarcoma. J Pediatr Surg 2000 Feb;35(2):309-16



Pubmed ID




Scopus ID

2-s2.0-12944273553   86 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.

Author List

Spunt SL, Lobe TE, Pappo AS, Parham DM, Wharam MD Jr, Arndt C, Anderson JR, Crist WM, Paidas C, Wiener E, Andrassy RJ, Schwartz CL


Cindy L. Schwartz MD, MPH Chief, Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Biliary Tract Neoplasms
Biliary Tract Surgical Procedures
Child, Preschool
Combined Modality Therapy
Retrospective Studies
Treatment Outcome
jenkins-FCD Prod-461 7d7c6113fc1a2757d2947d29fae5861c878125ab