Undifferentiated Embryonal Sarcoma of the Liver Successfully Treated With Chemotherapy and Liver Resection

Abstract

Undifferentiated embryonal sarcoma is the third most common malignant tumor of the liver in children, accounting for 13% of hepatic malignancies in this age group. It has been considered an aggressive neoplasm with very poor prognosis until the late 1980s, when long-term survivors were reported after multiagent chemotherapy followed by resection. We, herein, report two pediatric cases of undifferentiated embryonal sarcoma treated successfully with surgical resection after neoadjuvant chemotherapy based on therapy used in childhood soft tissue sarcomas and in childhood hepatic malignancies. The first patient also had a concurrent cerebellar tumor (pilocytic astrocytoma), for which he first underwent craniotomy and resection, delaying the liver tumor resection by 10 weeks. They are alive and tumor free at 48 months (case no. 1) and 18 months (case no. 2) following neoadjuvant chemotherapy and liver resection.

Management of undifferentiated embryonal sarcoma of the liver in children: A case series and management review

“…Subsequent articles suggest that the use of multimodal therapy has significantly improved prognosis. [6][7][8] This improvement in outcome may be related to a modern multidisciplinary approach, including potent combinations of neo-adjuvant and adjuvant chemotherapy, radical surgical resection and radiotherapy, and improved supportive care.…”

“…[7][8][9][10][11][12][13][14][15][16][17][18][19][20] Liver transplantation has been performed for non-resectable tumours, [21] or for recurrent disease after previous resection, and has been suggested to offer more likelihood of cure than adding radiation. [22] Five such patients have been documented with claimed disease-free survival from 18 months to 5 years.…”

“…[6] Early literature reports a poor prognosis with limited survival, and long-term survival rates are difficult to assess due to relatively short follow-up. Subsequent reports suggest significantly improved prognosis with the use of multimodal therapy, [6][7][8] which may be secondary to one of many factors. Some units favour a sequence of neo-adjuvant chemotherapy, followed by surgery and adjuvant chemotherapy.…”

Undifferentiated Embryonal Sarcoma of the Liver in an Adult: The Verification of the High Growth Rate in the Tumor

“…But recent authors report use of multiagent, adjuvant, or neoadjuvant chemotherapy followed by resection offer the best long-term results and possibly a cure (9). UES was known high malignant tumor and the fact that the tumor grows fast have been mentioned in several reports (2).…”

Primary undifferentiated embryonal sarcoma of the liver misdiagnosed as hydatid cyst in a child: case report and review of the literature

“…However, many authors suggest that multiagent, adjuvant or neo-adjuvant chemotherapy followed by resection offers the best long-term results and possibly a cure (13). Patients who underwent complete resection followed by adjuvant chemotherapy had significantly better survival rates compared with patients who underwent surgical resection alone.…”