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

Area of Science:

• Hepatobiliary Malignancies
• Pediatric Oncology
• Surgical Oncology

Background:

• Undifferentiated embryonal sarcoma of the liver (UESL) is a rare liver cancer predominantly affecting children.
• Clinicopathological characteristics and treatment outcomes in adults with UESL are less understood compared to pediatric cases.

Purpose of the Study:

• To compare the clinicopathological features and survival outcomes of pediatric and adult patients diagnosed with UESL.
• To identify prognostic factors influencing mortality in UESL across different age groups.

Main Methods:

• Retrospective analysis of 82 children and 41 adults with UESL from the National Cancer Database (2004-2015).
• Comparison of demographic, tumor, treatment, and survival data between pediatric and adult cohorts.
• Multivariable Cox regression analysis to determine independent predictors of mortality.

Main Results:

• No significant differences in tumor size, metastasis, surgical treatment, or margin status between children and adults.
• Children received chemotherapy more frequently (92.7% vs. 65.9%, p < 0.001).
• Children exhibited superior 5-year overall survival (84.4% vs. 48.2%, p < 0.001); adulthood was an independent risk factor for mortality.

Conclusions:

• Multimodal therapy, including surgery and chemotherapy, leads to favorable long-term survival in pediatric UESL.
• Adults with UESL experience poorer survival, suggesting potential differences in disease biology or treatment efficacy.
• Further refinement of treatment strategies for adult UESL is warranted to improve outcomes.