A case of undifferentiated embryonic liver sarcoma mimicking cystic hydatid disease in an endemic region of the world

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Abstract

Undifferentiated embryonic liver sarcoma (UELS) is a rare highly malignant neoplasm that predominantly occurs in children between 5 and 10 years of age. The typical radiologic appearance on ultrasound and computed tomography of UELS shows a large septated mass having combined cystic and solid components. These radiographic features, however, are not specific to UELS and are shared by other more common and benign diseases of the liver. For example, cystic hydatid disease (CHD), caused by larvae of the Echinococcus tapeworm, is the most common indication for hepatic operations in children residing in endemic regions of the world. Undifferentiated embryonic liver sarcoma and CHD are 2 diseases that share overlapping presenting features of patient age, symptoms, and radiologic appearance. Surgeons who operate in Echinococcal endemic regions must be aware that not all hepatic cystic masses are necessarily CHD and may be other more rare malignant diseases such as UELS.

Section snippets

Case report

A 7-year-old man was admitted to our hospital emergency department with acute abdominal pain. A physical examination revealed abdominal tenderness localized in the right upper quadrant, nausea, and vomiting. Laboratory studies showed normal serum bilirubin levels and mildly elevated aspartate aminotransferase (42 UI/L), alkaline phosphates (72 UI/L), and glutamic-pyruvic transaminase (53 UI/L) serum levels. Abdominal US showed a 6 × 5 cm cystic lesion with septation and echogenic material in

Discussion

Malignant primary hepatic tumors make up approximately 1% of pediatric tumors in the US [17]. Hepatoblastoma (HB) and hepatocellular carcinoma (HCC) are, by far, the most common malignant primary hepatic tumors in childhood [11]. Sarcomas are the third most common malignant primary hepatic tumors, comprising approximately 6% of hepatic malignancies in childhood [18]. For example, Moore et al [8] reported 194 cases of malignant primary hepatic tumors in children. Of these patients, 58% had HB,

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    None of the authors has a commercial interest, financial interest, and/or other relationship with manufacturers of pharmaceuticals, laboratory supplies, and/or medical devices or with commercial providers of medically related services.

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