undifferentiated embryonal sarcoma of the liver

undifferentiated embryonal sarcoma of the liver

A rare tumour usually seen in young children (typically under age 10), in whom it is the third most common liver tumour.

Clinical findings
Usually asymptomatic in children; adults often have right upper quadrant pain, an abdominal mass (usually affecting the right lobe of the liver), anorexia and intermittent fever.

Imaging
Cystic mass by ultrasonography, CT, MRI.
 
Managment
Resection plus pre- and/or postoperative systemic chemotherapy, liver transplant.

Prognosis
While distant metastasis is rare, the prognosis is usually poor. Long-term survival post complete resection; chemotherapy claims up to 71% survival at 20 years in children.
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References in periodicals archive ?
Management of undifferentiated embryonal sarcoma of the liver in children: A case series and management review.
At just 14 the teenager was believed to be only the second person in the country to be diagnosed with undifferentiated embryonal sarcoma of the liver.
Undifferentiated embryonal sarcoma of the liver (UESL) is a rare hepatic neoplasm that occurs mainly in children.
Undifferentiated embryonal sarcoma of the liver is the third most common hepatic malignancy in children <15 years of age.

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