Biliary tract embryonal rhabdomyosarcoma

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 Bilirubintotal serum bilirubin 14 mg/dLand Directdirect bilirubin 13 mg/dL.

The most common cause of benign obstructive jaundice in childhood arecholedochal 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.

There is a mixed echogenicity ill-defined mass lesion located in the portahepatis measuredporta hepatis measuring 5 x 4 cm, it shows increased blood flow on color Doppler mode. The mass encasingencases the hepatic artery, portal vein and extra hepaticextrahepatic biliary tree, causing intra hepaticintrahepatic dilatation of the biliary tree. Multiple enlarged portahepatisporta hepatis lymph nodes.

TISSUE ORIGINTissue origin: 1- Omentum. omentum. 2- Porta. porta hepatis lymph node. 3- Porta. porta hepatis mass. 

DIAGNOSISDiagnosis: Omentum, porta hepatis lymph node and porta hepatis mass; biopsies: Consistent with embryonal rhabdomyosarcoma. The tumor cells are positive for Desmindesmin, Myogenin (Focalmyogenin (focal) and Glypicanglypican-3. They are negative for Panpan-CK, SALL4 and Heppar1heppar1 immunostains.