Abstract
BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare liver tumor accounting for 6-13% of primary liver tumors. Accurate preoperative diagnosis is difficult, with a rather high misdiagnosis rate. Herein, we reported a very large UESL treated with anatomical resection. Our case is amongst the largest pediatric UESLs in the literature. CASE PRESENTATION: Herein, we report a 13-year-old girl presenting with right upper quadrant abdominal pain, postprandial vomiting, and abdominal distention, in which radiographic imaging demonstrated a huge UESL (28 × 20 × 12 cm). The patient was treated with partial hepatectomy and the 5 kg tumor was removed. The patient was discharged in good condition, with no significant complaints in her follow-up. CONCLUSIONS: Although different treatment strategies have been reported for UESL cases, anatomical resection is still the main treatment approach, especially for large tumors.