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

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.