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Childhood Liver Cancer Treatment (PDQ®): Treatment - Health Professional Information [NCI] - General Information

Table 1. Risk Factors Associated With Hepatoblastoma and Hepatocellular Carcinoma continued...

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.[56] Many UESL contain diverse elements of mesenchymal cell maturation, such as smooth muscle and fat. Undifferentiated sarcomas and small cell undifferentiated hepatoblastomas should be examined for loss of INI1 expression by immunohistochemistry to help rule out rhabdoid tumor of the liver.

It is important to make the diagnostic distinction between UESL and biliary tract rhabdomyosarcoma because they share some common clinical and pathologic features but treatment differs between the two, as shown in Table 2.[57] (Refer to the PDQ summary on Childhood Rhabdomyosarcoma Treatment for more information.)

Table 2. Diagnostic Differences Between Undifferentiated Embryonal Sarcoma of the Liver and Biliary Tract Rhabdomyosarcomaa

Undifferentiated Embryonal Sarcoma of the LiverBiliary Tract Rhabdomyosarcoma
a Adapted from Nicol et al.[57]
Age at DiagnosisMedian age 10.5 yMedian age 3.4 y
Tumor LocationOften arises in the right lobe of the liverOften arises in the hilum of the liver
Biliary ObstructionUnusualFrequently; jaundice is a common presenting symptom
TreatmentSurgery and chemotherapySurgery (usually biopsy only), radiation therapy, and chemotherapy

It has been suggested that some UESLs arise from mesenchymal hamartomas of the liver, which are large benign multicystic masses that present in the first 2 years of life.[57] Strong clinical and histological evidence suggest that UESL can arise within preexisting mesenchymal hamartomas of the liver. In a report of 11 cases of UESL, five arose in association with mesenchymal hamartomas of the liver, and transition zones between the histologies were noted.[58] Many mesenchymal hamartomas of the liver have a characteristic translocation with a breakpoint at 19q13.4 and several UESLs have the same translocation.[59,60] Some UESLs arising from mesenchymal hamartomas of the liver may have complex karyotypes not involving 19q13.4.

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 of the tumor. Clinical diagnosis may be made without biopsy based on tumor imaging of the liver associated with extremely high serum beta-hCG levels and normal AFP levels for age.[39]

Epithelioid Hemangioendothelioma

Epithelioid hemangioendothelioma is a rare vascular cancer that occurs in the liver and other organs. (Refer to the Hemangioendothelioma section in the PDQ summary on Childhood Soft Tissue Sarcoma Treatment for more information.)


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Last Updated: February 25, 2014
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