undifferentiated embryonal sarcoma of the liver


Also found in: Acronyms.

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 ?
Imaging features of undifferentiated embryonal sarcoma of the liver: a series of 15 children.
Undifferentiated embryonal sarcoma of the liver: case report and literature survey.
A case of adult undifferentiated embryonal sarcoma of the liver successfully treated with right trisectionectomy: a case report.
Broniszczak et al., "Treatment of undifferentiated embryonal sarcoma of the liver in children--single center experience," Journal of Pediatric Surgery, vol.
Raza, "Undifferentiated embryonal sarcoma of the liver with an unusual presentation: case report and review of the literature," Journal of Gastrointestinal Oncology, vol.
Kim et al., "Undifferentiated embryonal sarcoma of the liver: US and CT findings," Pediatric Radiology, vol.
Takahashi et al., "Undifferentiated embryonal sarcoma of the liver: case report and literature survey," Journal of Hepato-Biliary-Pancreatic Surgery, vol.
Sorensen, "Undifferentiated embryonal sarcoma of the liver: results of clinical management in one center," Journal of Pediatric Surgery, vol.
Bedros et al., "Undifferentiated embryonal sarcoma of the liver successfully treated with chemotherapy and liver resection," Journal of Gastrointestinal Surgery, vol.
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.

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