Undifferentiated embryonal sarcoma of the liver in a child: A case report and review of the literature

Abstract

Over 200 cases of undifferentiated embryonal sarcoma of the liver (UESL) have been reported since 1978 when this disease was first described. In the present study, we describe a case of UESL in a 7-year-old female, whose initial symptoms included swelling in the upper abdomen and a palpable enormous irregular tumor. A magnetic resonance imaging (MRI) examination revealed a massive focal lesion in the right lobe of the liver. Hepatic malignant tumor with a high possibility of hepatoblastoma was diagnosed. The tumor was surgically removed and confirmed to be UESL by postoperative pathology and immunohistochemical staining analysis. The patient then received chemotherapy consisting of three cycles of epirubicin (20 mg, days 1–2) and cisplatin (15 mg, days 1–3). To date, the patient has survived for 22 months, and is currently in a good general condition without evidence of local metastasis or recurrence. Although UESL has a high malignancy and a poor prognosis, cases of long-term survival with improved diagnosis and therapy have recently been reported. Therefore, it has been proposed that UESL should not be considered as an hepatic tumor with a poor prognosis. Total resection with preoperative or postoperative radio-chemotherapy is currently considered to be the key approach to improving the survival rate.