undifferentiated embryonal sarcoma of the liver
undifferentiated embryonal sarcoma of the liverA rare tumour usually seen in young children (typically under age 10), in whom it is the third most common liver tumour.
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.
Cystic mass by ultrasonography, CT, MRI.
Resection plus pre- and/or postoperative systemic chemotherapy, liver transplant.
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.