Undifferentiated sarcoma of the liver: a rare pediatric tumor

Abstract

Undifferentiated embryonal sarcoma of the liver (UESL) is an uncommon hepatic tumor of mesenchymal origin recognized as a unique clinicopathologic entity since 1978. UESL has historically been considered an aggressive neoplasm with an unfavorable prognosis. Survival has improved using recent multimodal approaches, designed for patients with soft tissue sarcomas at other sites. Several small series have reported survival of up to 70% of children. We report a case of a 12-year-old boy from the United Arab Emirates who relapsed after complete surgical resection and was then successfully treated with re-resection followed by chemotherapy and radiotherapy. With a follow-up of 5 years, he is well and asymptomatic, and is leading a healthy life. This case emphasizes the fact that these poorly prognostic tumors may benefit from post-surgery chemotherapy. This case illustrates the improved survival of UESL patients following the multimodality therapy with a relatively long follow-up. This is the first case of UESL reported in this region of the world.