| Summary: | This study evaluates the clinicopathological characteristics and outcomes of children vs. adults with undifferentiated embryonal sarcoma of the liver (UESL). A retrospective analysis of 82 children (<18 years) and 41 adults (≥18 years) with UESL registered in the National Cancer Database between 2004–2015 was conducted. No between-group differences were observed regarding tumor size, metastasis, surgical treatment, margin status, and radiation. Children received chemotherapy more often than adults (92.7% vs. 65.9%; <i>p</i> < 0.001). Children demonstrated superior overall survival vs. adults (log-rank, <i>p</i> < 0.001) with 5-year rates of 84.4% vs. 48.2%, respectively. In multivariable Cox regression for all patients, adults demonstrated an increased risk of mortality compared to children (<i>p</i> < 0.001), while metastasis was associated with an increased (<i>p</i> = 0.02) and surgical treatment with a decreased (<i>p</i> = 0.001) risk of mortality. In multivariable Cox regression for surgically-treated patients, adulthood (<i>p</i> = 0.004) and margin-positive resection (<i>p</i> = 0.03) were independently associated with an increased risk of mortality. Multimodal treatment including complete surgical resection and chemotherapy results in long-term survival in most children with UESL. However, adults with UESL have poorer long-term survival that may reflect differences in disease biology and an opportunity to further refine currently available treatment schemas.
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