Aggressive surgery is unwarranted for biliary tract rhabdomyosarcoma,☆☆

Presented at the 30th Annual Meeting of the American Pediatric Surgical Association, Rancho Mirage, California, May 16-19, 1999.
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Abstract

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. J Pediatr Surg 35:309-316. Copyright © 2000 by W.B. Saunders Company.

Section snippets

Materials and methods

Among patients registered on IRS protocols (I, II, III, IV-P, and IV) from November 1972 through July 1998, 4,291 were eligible for evaluation. Twenty-five of these (0.5%) had biliary RMS and are the subject of this review. Biliary RMS includes tumors arising from the intrahepatic or extrahepatic biliary tree, gallbladder, cystic duct, and ampulla of Vater. Tumors arising elsewhere in the gastrointestinal tract, in the retroperitoneum, and undifferentiated sarcoma arising in the liver were

Clinical presentation

Twenty-five eligible patients with biliary RMS were treated on IRS protocols I, II, III, IV-P, and IV (Table 1).Sixteen patients were male, and 15 were white, 6 black, and 4 Hispanic. Median age at presentation was 3.4 years (range, 0.4 years to 9.6 years). As shown in Table 2, patients with biliary RMS were significantly younger than other patients with RMS, who had a median age of 6 years (range, birth to 21 years; P <.001).Common presenting symptoms were jaundice, abdominal pain, vomiting,

Discussion

This report reviews 25 consecutive cases of biliary RMS treated according to the Intergroup Rhabdomyosarcoma Study Group protocols between 1972 and 1998. We confirm prior reports3, 11, 12 that the classic presentation of biliary RMS is a young child (average age 3½ years) with jaundice and abdominal pain, often associated with abdominal distension, vomiting, and fever. Laboratory evaluation typically shows moderate to marked conjugated hyperbilirubinemia with mild hepatic transaminase

Acknowledgements

The authors thank Dr Harold Maurer for his involvement in this project; Julie Moore, IRSG Biopathology Center, for assistance in obtaining pathological reports for review; and Sherry Stephens for secretarial support.

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Supported in part by grants (CA-24507-21 and CA72989-02) from the National Cancer Institute.

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