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

Research output: Contribution to journalArticlepeer-review

Authors

  • Manasvi Upadhyaya
  • Patrick McKiernan
  • David Hobin
  • Deirdre A Kelly
  • Rachel Brown
  • And 6 others
  • Carla Lloyd
  • John Buckels
  • Alastair J W Millar
  • Jean deVille deGoyet
  • Darius F Mirza
  • Khalid Sharif

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.

Details

Original languageEnglish
Pages (from-to)2124-8
Number of pages5
JournalJournal of pediatric surgery
Volume45
Issue number11
Publication statusPublished - Nov 2010

Keywords

  • Adolescent, Angiography, Antineoplastic Agents, Biopsy, Child, Child, Preschool, Diagnosis, Differential, Female, Follow-Up Studies, Hepatectomy, Humans, Infant, Liver Neoplasms, Magnetic Resonance Imaging, Male, Retrospective Studies, Sarcoma, Time Factors, Tomography, X-Ray Computed, Treatment Outcome