Objective  To evaluate the clinical manifestation, imaging characteristics, and treatment plans of undifferentiated embryonal sarcoma of the liver (UESL) to provide bases for the diagnosis and treatment.

  Methods  Data of two UESL cases were obtained from The Affiliated Tumor Hospital of Guangxi Medical University, Naning, China. Information on relevant cases was retrieved from the CNKI, Wanfang, and VIP databases between January 1988 and June 2011. Clinical data of all the cases were analyzed.

  Results  A total of 120 UESL cases previously reported in China were included in this study. Most of the cases presented with an abdominal mass, with a low positive rate of hepatitis B surface antigen and alpha fetoprotein (17.72 % and 8.64 %). The average diameter of the tumors was 13.82 cm-4.90 cm. Imageological diagnosis revealed that 72.34 % of the UESL cases showed a solid hyperechogenieity in ultrasound, whereas 91.78 % of the cases demonstrated a cystic low density on CT scan. The recurrence rate was higher in the group with surgery than in the group with surgery plus chemotherapy (72.97 % and 30.77 %). However, no significant differences in recurrence time and survival curves were found between the two groups. The overall median survival time (95 % confidence interval) was 11 (5.4±16.6) months.

  Conclusion  Early diagnosis and prompt excision play an important role in the treatment and prognosis of UESL. Furthermore, postoperative adjuvant chemotherapy can reduce the risk of tumor recurrence and prolong the survival time for UESL patients.