Aggressive surgery is unwarranted for biliary tract rhabdomyosarcoma. J Pediatr Surg 2000 Feb;35(2):309-16
Date
02/29/2000Pubmed ID
10693686DOI
10.1016/s0022-3468(00)90030-7Scopus ID
2-s2.0-12944273553 (requires institutional sign-in at Scopus site) 114 CitationsAbstract
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 CLAuthor
Cindy L. Schwartz MD, MPH Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Biliary Tract NeoplasmsBiliary Tract Surgical Procedures
Child
Child, Preschool
Combined Modality Therapy
Female
Humans
Infant
Male
Reoperation
Retrospective Studies
Rhabdomyosarcoma
Treatment Outcome
