Format

Send to

Choose Destination
Pediatr Blood Cancer. 2017 Apr;64(4). doi: 10.1002/pbc.26272. Epub 2016 Oct 26.

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

Author information

1
Divisions of Pediatric Surgery and Surgical Research, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
2
Texas Children's Department of Surgery, Texas Children's Liver Tumor Center, Baylor College of Medicine, Houston, Texas.
3
Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas.
4
Outcomes & Impact Service, Department of Surgery, Texas Children's Hospital, Houston, Texas.
5
Divison of Pediatric Surgery, Department of Surgery, University of Alabama, Birmingham, Alabama.
6
Department of Surgery, Methodist Children's Hospital of South Texas, University of Texas Health Science Center - San Antonio, San Antonio, Texas.
7
Department of Surgery, John Wayne Cancer Institute at Providence St. Johns Health Center, Santa Monica, California.
8
Division of Pediatric General and Thoracic Surgery, Seattle Children's Hospital, Department of Surgery, University of Washington, Seattle, Washington.
9
Department of Surgery, Maine Children's Cancer Program, Tufts University, Portland, Maine.
10
Division of Pediatric Surgery, Primary Children's Hospital, University of Utah, Salt Lake City, Utah.

Abstract

OBJECTIVE:

To examine patient characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver (UESL) using a multi-institutional database.

SUMMARY BACKGROUND DATA:

UESL is a rare disease (incidence is one per million). Therefore, the current literature is mostly limited to small case series.

METHODS:

The National Cancer Database was queried for primary UESL diagnosed between 1998 and 2012.

RESULTS:

A 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.

CONCLUSION:

Surgical 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.

KEYWORDS:

National Cancer Database; embryonal sarcoma; hepatic sarcoma; liver cancer; liver sarcoma; undifferentiated embryonal sarcoma

PMID:
27781381
PMCID:
PMC5333454
DOI:
10.1002/pbc.26272
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for Wiley Icon for PubMed Central
Loading ...
Support Center