A Case of Long-Term Survivor after Surgical Resection for Undifferentiated Embryonal Sarcoma of the Liver in Adult / 한국간담췌외과학회지

ABSTRACT

An undifferentiated (embryonal) sarcoma of the liver (USL) is a rare and highly malignant hepatic neoplasm of mesenchymal origin. This tumor almost exclusively affects pediatric patients, with a poor prognosis. The highest incidence is noted in pediatric patients, usually from 5~10 years of age, but only 43 cases of USL have been reported in adults worldwide since it was first as a clinicopathological entity. With a USL in adults, most patients are known to have died within 1 year of diagnosis. The absence of specific symptoms, rapid tumor growth, normality of common tumor markers and the consequential delay in diagnosis often result in significant enlargement of the tumor, with a poor prognosis. Various therapeutic modalities have been attempted in adult patients, but only a few long-term survivors have been reported. Herein, our experience of a 42-year-old female patient who is still alive, 55 months after the first resection for a USL is reported.