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.
Mentioned in ?
References in periodicals archive ?
The term undifferentiated embryonal sarcoma of the liver (UESL) was introduced by Stocker and Ishak (1) in 1978 to describe groups of mesenchymal tumor in the liver that did not show evidence of differentiation.
Undifferentiated embryonal sarcoma of the liver is often mistaken for a benign hepatic lesion because of its cystic appearance.
Undifferentiated embryonal sarcoma of the liver usually occurs as a single and well-circumscribed lesion grossly.
Undifferentiated embryonal sarcoma of the liver is often considered a malignant evolution of MH.
Undifferentiated embryonal sarcoma of the liver has an exhaustive differential diagnosis list.
Undifferentiated embryonal sarcoma of the liver mimicking acute appendicitis: case report and review of the literature.
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.
Nicol K, Savell V, Moore J, Teot L, Spunt SL, Qualman S; Children\'s Oncology Group, Soft Tissue Sarcoma Committee Distinguishing undifferentiated embryonal sarcoma of the liver from biliary tract rhabdomyosarcoma: a children oncology group study.
Undifferentiated embryonal sarcoma of the liver (UESL) is a rare hepatic neoplasm that occurs mainly in children.
Undifferentiated embryonal sarcoma of the liver successfully treated with chemotherapy and liver resection.
Undifferentiated embryonal sarcoma of the liver is the third most common hepatic malignancy in children <15 years of age.

Full browser ?