Characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver: A report from the National Cancer Database

Abstract

ObjectiveTo examine patient characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver (UESL) using a multi‐institutional database.Summary Background DataUESL is a rare disease (incidence is one per million). Therefore, the current literature is mostly limited to small case series.MethodsThe National Cancer Database was queried for primary UESL diagnosed between 1998 and 2012.ResultsA total of 103 patients (<18 years) were identified. The 5‐year overall survival of the entire group was 86%. The best outcomes were seen in children who had tumors smaller than 15 cm and were able to undergo surgical resection with or without chemotherapy. Margin status did not appear to significantly affect survival. The most common type of resection was hemihepatectomy (37%), followed by sectionectomy (10%) and trisectionectomy (10%). Orthotopic liver transplant was performed in 10 children, all of whom survived to 5 years.ConclusionSurgical resection with or without chemotherapy should be the mainstay of treatment in children with UESL, and is associated with very favorable outcomes. Negative surgical margins were not associated with improved survival. Orthotopic liver transplantation may be a viable method of attaining local control in tumors, which would otherwise be unresectable.

Undifferentiated Embryonal Sarcoma of the Liver Presents as a Molecular Mimic of Parasitic Infection

“…Undifferentiated embryonal sarcoma of the liver (UESL) is an extremely rare but highly malignant primary hepatic cancer of mesenchymal origin. It is primarily found in children aged 6-10 years, but is extremely rare with only 103 children being diagnosed between 1998 and 2012, with an overall five-year survival of 86% [4]. Early diagnosis is associated with better prognosis, making rapid diagnosis and treatment vital [4].…”

“…It is primarily found in children aged 6-10 years, but is extremely rare with only 103 children being diagnosed between 1998 and 2012, with an overall five-year survival of 86% [4]. Early diagnosis is associated with better prognosis, making rapid diagnosis and treatment vital [4]. UESL typically presents as an abdominal mass with or without pain.…”

Clinical and imaging characteristics of primary hepatic sarcomatoid carcinoma and sarcoma: a comparative study

“…The current study concluded that PHSCs mainly showed ring hyper-enhancement on the AP, followed a washout on the later phase. It was reported that the diverse tissue compositions of PHSC determine its enhancement pattern (6). The PHSCs, especially the S-HCCs, were characterized by peripheral viable cancerous tissue (viable cells, higher microvascular densities and relatively less brous tissue) and central necrosis.…”

“…The PHSC and PHS have many overlapping features in imaging as well as clinical and pathological ndings (4)(5)(6), but their treatment modalities may be different, even though the most optimal therapy still awaits further evidence, due to the dearth of available information caused by their rarity. In the group of PHSs, recent studies have suggested that a combination of surgery and pre-or post-surgical chemotherapy can substantially improve clinical outcomes of the UES.…”

The genomic landscape of undifferentiated embryonal sarcoma of the liver is typified by C19MC structural rearrangement and overexpression combined with TP53 mutation or loss

“…Undifferentiated embryonal sarcoma of the liver (UESL) is an aggressive primitive malignancy. It occurs predominantly in children with a peak age incidence of 6-10 years and equally across gender [1]. UESL mostly arises from the right lobe of the liver [2], presenting with nonspecific symptoms of abdominal pain, fever, nausea and anorexia.…”