Aggressive surgery is unwarranted for biliary tract rhabdomyosarcoma☆,☆☆
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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