Rhabdomyosarcomas (biliary tract)
Rhabdomyosarcomas of the biliary tract are rare tumours, usually identified in children, with a very poor prognosis. They are usually grouped under botryoid rhabdomyosarcomas.
For a general discussion of this type of tumour, please refer to the article on rhabdomyosarcomas.
Rhabdomyosarcomas are considered the most common biliary tract tumours in children 4. The median age of presentation is 3 years with a slight male preponderance 3.
The most common clinical features are jaundice and abdominal distention, with pain, vomiting, and fever being less frequent. Elevation in liver transaminases and bilirubin is often present.
Rhabdomyosarcomas of the biliary tract most commonly arises from the common bile duct 1, but it can arise from almost anywhere along the biliary tree including liver, intrahepatic and extrahepatic biliary ducts, gallbladder, or ampulla. It has also been reported to arise from hepatic and choledochal cysts.
Ultrasound imaging will demonstrate intrahepatic biliary ductal dilatation and an intraductal mass. Cystic areas may be seen in larger tumours and may represent areas of necrosis3. Because of this, it can have a radiologic appearance similar to a choledochal cyst, especially if there is no local invasion.
CT may show a heterogeneous or hypoattenuating mass with biliary ductal dilatation. CT may be the best modality for surveillance of recurrence.
MRI has advantages over other modalities because of its ability to define the extent of disease and relationship to the hepatic vasculature.
If the tumour is large and grows into the liver, it may be difficult to distinguish from other primary liver masses 3.
- 1. Donnelly LF, Bisset GS, Frush DP. Diagnosis please. Case 2: Embryonal rhabdomyosarcoma of the biliary tree. Radiology. 1998;208 (3): 621-3. Radiology (citation) - Pubmed citation
- 2. Kransdorf Mark J and Mark D Murphey. Imaging of Soft Tissue Tumors. Second Edition. Lippincott Williams & Wilkins, 2006. ISBN: 0781747716.
- 3. McGahan JP, Goldberg BB. Diagnostic ultrasound. Informa Health Care. (2008) ISBN:1420069780. Read it at Google Books - Find it at Amazon
- 4. Zampieri N, Camoglio F, Corroppolo M et-al. Botryoid rhabdomyosarcoma of the biliary tract in children: a unique case report. Eur J Cancer Care (Engl). 2006;15 (5): 463-6. Eur J Cancer Care (Engl) (full text) - doi:10.1111/j.1365-2354.2006.00683.x - Pubmed citation
- 5. Friedburg H, Kauffmann GW, BöHm N et-al. Sonographic and computed tomographic features of embryonal rhabdomyosarcoma of the biliary tract. Pediatr Radiol. 1985;14 (6): 436-8. Pubmed citation
- 6. Roebuck DJ, Yang WT, Lam WW et-al. Hepatobiliary rhabdomyosarcoma in children: diagnostic radiology. Pediatr Radiol. 1998;28 (2): 101-8. Pubmed citation
- 7. Sanz N, De Mingo L, FlóRez F et-al. Rhabdomyosarcoma of the biliary tree. Pediatr. Surg. Int. 1997;12 (2-3): 200-1. Pubmed citation