Outcome of patients with undifferentiated embryonal sarcoma of the liver treated according to European soft tissue sarcoma protocols

Guérin F, Martelli H, Rogers T, Zanetti I, Terwisscha van Scheltinga S, De Corti F, Guillen Burrieza G, Minard-Colin V, Orbach D, van Noesel MM, Karanian M, Dávila Fajardo R, Merks JHM, Ferrari A, Bisogno G, Pediatr Blood Cancer. 2023 Jul;70(7):e30374. doi: 10.1002/pbc.30374. Epub 2023 Apr 21.

Highlights of the paper:

Neoadjuvant chemotherapy for pediatric patients with UESL increases the quality of surgery.

Five-year overall and event-free survivals were 90.1% and 89.1% respectively.

Alkylating agents should not be omitted or reduced.

The role of anthracyclines and radiotherapy for localized disease remains unclear.

Summary: Our study assessed the outcomes of 65 pediatric patients with Undifferentiated Embryonal Sarcoma of the Liver treated according to successive European malignant mesenchymal tumor trials. This study demonstrated excellent outcomes with 90% overall survival and 89% event free-survival at 5 years with an association of chemotherapy and surgery. Neoadjuvant chemotherapy improved the quality of surgery. Eight events consisted of 4 local (2 were associated with invaded surgical margins), 3 metastatic relapses (two were associated with a reduced alkylating regimen), and one-second tumor. There was no statistical association between invaded margins, use of radiotherapy or anthracycline and these events.