Undifferentiated Embryonal Sarcoma of the Liver in Children Versus Adults: A National Cancer Database Analysis

• Main Authors: Ioannis A. Ziogas, Irving J. Zamora, Harold N. Lovvorn III, Christina E. Bailey, Sophoclis P. Alexopoulos
• Format: Article
• Language: English
• Published: MDPI AG 2021-06-01
• Series: Cancers
• Subjects: embryonal sarcoma; liver sarcoma; hepatic sarcoma; liver cancer; National Cancer Database
• Online Access: https://www.mdpi.com/2072-6694/13/12/2918

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.