[Surgical diagnosis and treatment of adult huge undifferentiated embryonal sarcoma of the liver].

Objective

To investigate the clinical characteristics of adult undifferentiated embryonal sarcoma of the liver (UESL).

Methods

A retrospective analysis was performed on the clinical data of 5 patients with UESL who underwent surgical resection and were pathologically confirmed from January 2005 to December 2020 at the First and the Second Affiliated Hospital of Anhui Medical University. All the patients were female aged from 49 to 77 years old. Preoperative CT showed a solid cystic mass with low density and a slight density of cord like septum. Imaging findings were misdiagnosed as hepatocellular carcinoma or cystadenocarcinoma. CA125 was higher in 3 patients,and AFP in all patients was normal.

Results

All patients were treated by surgery. The mean diameter of tumor was 20.2 cm (range:15.0 to 30.0 cm). All five patients had vimentin expression in immunohistochemistry. Three cases underwent complete resection of the tumor and achieved R0 resection,2 of them had tumor free survival until the end of the follow-up (89 and 55 months),the other 1 case died from renal cell carcinoma 158 months later. The remaining 2 cases were radically resected,but the tumors were ruptured during operation,and relapsed after 2 months and 19 months respectively. The overall survival was 3 and 26 months respectively.

Conclusions

Radical hepatectomy is the first choice for treatment of UESL. Intraoperative tumor rupture should be avoided and implant metastasis is a major factor affecting the prognosis of UESL.