Outcomes of primary liver cancer in children: an appraisal of experience.

INTRODUCTION: Hepatoblastoma (HB) and hepatocellular carcinoma (HCC) are the most common primary liver cancers in children. Recent advances in management of pediatric liver cancer have improved disease-specific survival (DSS). This is a review of our experience with childhood liver malignancy over the past 3 decades. MATERIALS AND METHODS: A retrospective chart review from 1975 to 2005 identified patients who were 18 years old or younger with a histologically confirmed diagnosis of primary liver cancer. Patients were staged according to the Children's Cancer Group and Pediatric Oncology Group (CCG/POG) system. Patients were followed up prospectively through clinic visits and mail correspondence. Standard statistical methods were used for comparison, risk, and survival analyses. RESULTS: Fifty-two patients were confirmed to have primary liver cancers, where 24 (46%) patients had HB, 22 (42%) had HCC, 3 (6%) had sarcomas, and 3 (6%) had other histologies. Mean ages at presentation for HB and HCC were 3.2 and 13.1 years old, respectively. The most common presentations were abdominal mass (67%) and pain (40%). Most patients underwent major liver resection (n = 45, 87%), including: lobectomy (n = 25, 48%), and trisegmentectomy (n = 11, 21%). Three patients underwent liver transplantation (n = 3, 6%) for advanced local disease. Forty-five (87%) received primary or neoadjuvant and/or adjuvant chemotherapy. Patients had the following CCG/POG stages: I (n = 31, 60%), II (n = 6, 11.5%), III (n = 9, 17%), and IV (n = 6, 11.5%). Complete gross resection (stage I and II) was achieved in 37 (71%) patients. The perioperative mortality and morbidity rates were 0% and 29%, respectively. Patients with complete resection had significantly better 5-year DSS and median survival compared with incomplete gross resection: 62% vs 9% and 216 vs 18 months, P < .001. Patients treated during the period 1995-2005 had better 5-year DSS and median survival compared with those treated during 1975-1994: 68% vs 32% and 117 vs 27 months, P = .032. All 3 patients who underwent transplantation for conventionally unresectable disease are alive without disease recurrence (follow-up period, 1-15 years). CONCLUSION: Complete resection of the pediatric primary liver tumors remains the cornerstone of treatment to achieve cure. Major liver resection can be performed with minimal perioperative mortality and morbidity. Patients with HB appeared to have better survival compared with patients with HCC, and there was significant improvement in the DSS of children treated in the recent decade. Liver transplantation in conjunction with chemotherapy may have an increasing role in the management of locally advanced primary liver cancers.