Primary hepatic sarcomas in children - A single-center experience over 19 years

Manasvi Upadhyaya, Patrick McKiernan, David Hobin, Deirdre A. Kelly, Rachel Brown, Carla Lloyd, John Buckels, Alastair J W Millar, Jean Deville Degoyet, Darius F. Mirza, Khalid Sharif

Research output: Contribution to journalArticlepeer-review

Abstract

Background/ Purpose: Primary hepatic sarcomas are rare and account for about 13% of primary hepatic neoplasms. There are few reported series of pediatric hepatic sarcomas, and the aim was to review our experience. Methods: A retrospective analysis of cases managed from 1988 to 2007 by the pediatric liver unit in Birmingham, UK, was conducted. Results: Nineteen children were identified. These presented with sudden abdominal pain (n = 6), obstructive jaundice (n = 3), incidental mass (n = 3), and chronic pain/distension (n = 3). Vascular involvement was identified in 3, and 6 had pulmonary metastases. Three patients had primary resection, and 3 only a biopsy. Thirteen had a biopsy followed by chemotherapy and resection. Surgery included extended hepatectomy (n = 11), hepatectomy (n = 3), and nonanatomical resections (n = 2). There was 1 major intraoperative complication. Median inpatient stay was 7 days. One biliary leak developed 4 weeks postoperatively. Five of the 16 patients who underwent resection of the primary tumor died. Eleven were alive at a median follow-up of 3 years. Conclusion: This is a challenging group of patients. Local control remains pivotal to successful treatment. Good results can be achieved in a specialist center with multidisciplinary approach.

Original languageEnglish
Pages (from-to)2124-2128
Number of pages5
JournalJournal of Pediatric Surgery
Volume45
Issue number11
DOIs
Publication statusPublished - Nov 2010

Keywords

  • Pediatric liver unit
  • Pediatric tumors
  • Primary hepatic sarcomas

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

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