A Case of Adult Undifferentiated Embryonal Sarcoma of the Liver Successfully Treated with Right Trisectionectomy: a Case Report

Background: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant mesenchymal tumor that usually occurs in children and is rarely diagnosed in adults.

Case Presentation: Here, we describe the case of a 65-year-old woman who presented with a huge cystic lesion in the liver. Laboratory studies performed on admission showed modest inflammation, poor nutrition, and elevated levels of total bilirubin, alkaline phosphatase, and γ-glutamyl transferase. Computed tomography showed a well-defined, heterogeneous tumor with multiple cysts involving the right lobe and the medial segment of the liver, with a maximum diameter of 16 cm. Positron emission tomography/computed tomographic scans showed the uptake of 2-(fluorine-18)-fluoro-2-deoxy-D-glucose in a part of the cyst. The patient was diagnosed with mucinous cystadenocarcinoma or sarcoma of the liver and underwent right trisectionectomy. Histopathological studies revealed that the tumor was composed of pleomorphic and polynuclear dyskaryotic cells with eosinophilic globules in the cytoplasm. Mesenchymal hamartoma-like tissue was observed in the peripheral part of the tumor. Immunohistochemical analyses showed the tumor stained with vimentin, α-smooth muscle actin, desmin, α1-antitrypsin, and α1-antichymotripsin. Therefore, a histological diagnosis of UESL was made. Eighteen months following treatment, two recurrent tumors in the remnant liver were detected and resection of the recurrent tumors was performed.

Conclusions: A UESL should be considered in the differential diagnosis of large cystic hepatic lesions. Although the prognosis of UESL is extremely unfavorable, aggressive surgical resection should be the most important factor for ensuring long-term survival.