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Characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver: A report from the National Cancer Database.
Shi, Yan; Rojas, Yesenia; Zhang, Wei; Beierle, Elizabeth A; Doski, John J; Goldfarb, Melanie; Goldin, Adam B; Gow, Kenneth W; Langer, Monica; Meyers, Rebecka L; Nuchtern, Jed G; Vasudevan, Sanjeev A.
Afiliação
  • Shi Y; Divisions of Pediatric Surgery and Surgical Research, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
  • Rojas Y; Texas Children's Department of Surgery, Texas Children's Liver Tumor Center, Baylor College of Medicine, Houston, Texas.
  • Zhang W; Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas.
  • Beierle EA; Divisions of Pediatric Surgery and Surgical Research, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
  • Doski JJ; Texas Children's Department of Surgery, Texas Children's Liver Tumor Center, Baylor College of Medicine, Houston, Texas.
  • Goldfarb M; Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas.
  • Goldin AB; Outcomes & Impact Service, Department of Surgery, Texas Children's Hospital, Houston, Texas.
  • Gow KW; Divison of Pediatric Surgery, Department of Surgery, University of Alabama, Birmingham, Alabama.
  • Langer M; Department of Surgery, Methodist Children's Hospital of South Texas, University of Texas Health Science Center - San Antonio, San Antonio, Texas.
  • Meyers RL; Department of Surgery, John Wayne Cancer Institute at Providence St. Johns Health Center, Santa Monica, California.
  • Nuchtern JG; Division of Pediatric General and Thoracic Surgery, Seattle Children's Hospital, Department of Surgery, University of Washington, Seattle, Washington.
  • Vasudevan SA; Division of Pediatric General and Thoracic Surgery, Seattle Children's Hospital, Department of Surgery, University of Washington, Seattle, Washington.
Pediatr Blood Cancer ; 64(4)2017 04.
Article em En | MEDLINE | ID: mdl-27781381
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.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Sarcoma / Bases de Dados Factuais / Neoplasias Embrionárias de Células Germinativas / Neoplasias Hepáticas Tipo de estudo: Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male / Newborn Idioma: En Ano de publicação: 2017 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Sarcoma / Bases de Dados Factuais / Neoplasias Embrionárias de Células Germinativas / Neoplasias Hepáticas Tipo de estudo: Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male / Newborn Idioma: En Ano de publicação: 2017 Tipo de documento: Article