Biliary tract embryonal rhabdomyosarcoma

Case contributed by Hazem M Almasarei


One of twins, previously healthy, presented with abdominal pain and diarrhea with changes in urine and stool color, then yellowish discoloration of skin and sclera. Labs: total serum bilirubin 14 mg/dL and direct bilirubin 13 mg/dL.

Patient Data

Age: 4 years
Gender: Female

There is a mixed echogenicity ill-defined mass lesion located in the porta hepatis measuring 5 x 4 cm, it shows increased blood flow on color Doppler. The mass encases the hepatic artery, portal vein and extrahepatic biliary tree, causing intrahepatic dilatation of the biliary tree. Multiple enlarged porta hepatis lymph nodes.

There is an ill-defined mass lesion in the porta hepatis encasing the adjacent structures (hepatic artery, portal vein and common bile duct) causing moderate dilatation of the intrahepatic biliary tree and associated with lymphadenopathy. 

Tissue origin: 1. omentum. 2. porta hepatis lymph node. 3. porta hepatis mass. 

Diagnosis: Omentum, porta hepatis lymph node and porta hepatis mass; biopsies: Consistent with embryonal rhabdomyosarcoma. The tumor cells are positive for desmin, myogenin (focal) and glypican-3. They are negative for pan-CK, SALL4 and heppar1 immunostains.

Case Discussion

The most common cause of benign obstructive jaundice in childhood are choledochal cysts, while the most common malignant cause of obstructive jaundice in childhood is rhabdomyosarcoma. However in this case, the other differential diagnosis is lymphoma (homogeneous mass enhancement and associated with lymphadenopathy), while rhabdomyosarcoma mainly shows heterogeneous enhancement.

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