Case Report

Embryonal rhabdomyosarcoma of the biliary tree in a paediatric patient – A rare cause of obstructive jaundice

Denny Mathew, Heliodora de Lima, Nasreen Mahomed
South African Journal of Radiology | Vol 23, No 1 | a1662 | DOI: | © 2019 Denny Mathew, Heliodora de Lima, Nasreen Mahomed | This work is licensed under CC Attribution 4.0
Submitted: 04 September 2018 | Published: 10 January 2019

About the author(s)

Denny Mathew, Department of Radiology, University of the Witwatersrand, South Africa
Heliodora de Lima, Helen Joseph Hospital, Johannesburg, South Africa; and Rahima Moosa Mother and Child Hospital, Johannesburg, South Africa
Nasreen Mahomed, Department of Radiology, University of the Witwatersrand, South Africa; and Department of Radiology, Rahima Moosa Mother and Child Hospital, Johannesburg, South Africa; and South African Society of Paediatric Imaging, Cresta, South Africa


Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in the paediatric age group, ranking fourth in frequency after central nervous system tumours, neuroblastomas and nephroblastomas. Embryonal RMS of the biliary tree is considered a rare entity, with the most common clinical presentation being that of obstructive jaundice. We present the case of a 4-year-old boy who presented with hepatomegaly and obstructive jaundice. Biochemically, there was evidence of elevated ductal enzymes with conjugated hyperbilirubinaemia. The magnetic resonance imaging (MRI) features were consistent with a biliary RMS with the differential diagnosis of a choledochal cyst initially included based on the computed tomography images. The diagnosis of embryonal biliary RMS was later confirmed on histology. This case illustrates the importance of considering malignant aetiologies in paediatric cases of obstructive jaundice, as this entity is infrequently described in the literature and may mimic the appearance of a choledochal cyst. The demonstration of enhancement of intraductal material within the biliary tree on MRI and the presence of arterial waveforms within the intraductal mass on ultrasound assists in the differentiation between biliary RMS and a choledochal cyst.


biliary rhabdomyosarcoma; choledochal cyst; obstructive jaundice


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Crossref Citations

1. Embryonal Rhabdomyosarcoma of the Biliary Tree as a Differential in a Paediatric Patient Presenting with Biliary Dilatation: Not Always a Choledochal Cyst
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doi: 10.15388/Amed.2021.29.1.2