Undifferentiated Embryonal Sarcoma of the Liver
Undifferentiated embryonal sarcoma of the liver (UESL) is the third most common liver malignancy in children and adolescents, comprising 9% to 13% of liver tumors. It presents as an abdominal mass, often with pain or malaise, usually between the ages of 5 and 10 years. Widespread infiltration throughout the liver and pulmonary metastasis are common. It may appear solid or cystic on imaging, frequently with central necrosis. Distinctive features are characteristic intracellular hyaline globules and marked anaplasia on a mesenchymal background. Many UESL contain diverse elements of mesenchymal cell maturation, such as smooth muscle and fat.
It is important to make the diagnostic distinction between UESL and biliary tract rhabdomyosarcoma (BTR) as they share some common clinical and pathologic features but treatment differs between the two as shown in Table 1. (Refer to the PDQ summary on Childhood Rhabdomyosarcoma Treatment for more information.)
Table 1. Diagnostic Differences Between Undifferentiated Embryonal Sarcoma of the Liver and Biliary Tract Rhabdomyosarcomaa
a Adapted from 
|�||Undifferentiated Embryonal Sarcoma of the Liver||Biliary Tract Rhabdomyosarcoma|
|Age at Diagnosis||Median age 10.5 y||Median age 3.4 y|
|Tumor Location||Often arises in the right lobe of the liver||Often arises in the hilum of the liver|
|Biliary Obstruction||Unusual||Frequently; jaundice is a common presenting symptom|
|Treatment||Surgery and chemotherapy||Surgery, radiation therapy, and chemotherapy|
It has been suggested that some UESLs arise from mesenchymal hamartomas of the liver (MHL), which are large benign multicystic masses that present in the first 2 years of life. Strong clinical and histological evidence suggest that UESL can arise within a preexisting MHL. Many MHLs have a characteristic translocation with a breakpoint at 19q13.4 and several UESLs have the same translocation.[51,52]
Infantile Choriocarcinoma of the Liver
Choriocarcinoma of the liver is a very rare tumor that appears to originate in the placenta and presents with a liver mass in the first few months of life. Infants are often unstable due to hemorrhage from the tumor. Clinical diagnosis may be made without biopsy based on extremely high serum beta-hCG levels and normal AFP levels for age.
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