Surgical Experience of Giant Hepatoblastoma Involving the Inferior Vena Cava in Children: Report of 21 Cases of a Single Center

Background: Hepatic resection is the main treatment modality for giant Hepato Blastoma (HB), but operative treatment for giant Hepato Blastoma (HB) invading inferior vena cava is regarded as challenging because of its deep location in the liver and possibly worse prognosis especially in children. With the development of diagnostic technique, preoperative preparation, surgical technique, and postoperative management, some similar cases had a better prognosis after operation. The objective of our study is to report our experience in hepatectomy for giant Hepato Blastoma (HB) Invading Inferior Vena Cava (IVC) in children and to review the clinical features and survival rates retrospectively. Methods : A retrospective review of 21 patients who underwent operation for HB in children between January 1998 and December 2012 was performed. Clinical features were analyzed such as age, gender, symptoms, laboratory investigations, postoperative pathological results, operation technique, and intra-operative and postoperative complications etc. Survival and recurrence outcomes were analysed using Cox hazard models and the Kaplan-Meier method. Results : They included 14 boys and 7 girls ranging in age from 4 months to 168 months with a mean of 46±42.44months, the median duration of survival after resection was 36 months. Only one boy had abdominal pain and distension, and 20 cases without symptoms were determinated by chance. Most of the blood tests are normal excluding those for a–fetoprotein 773.84±400.05 (normal vs . abnormal, 3 vs . 18), platelet ranging from 201×10 9 /L to 910×10 9 /L (mean, 773.82±180.06×10 9 /L). Liver function was classified as Child-Pugh A for all patients. Four patients were positive for hepatitis B surface antigen. The tumor diameter ranged from 5.0cm to 20.0cm (mean, 12.38±3.99 cm). All the patients performed surgery successfully and two patients had received 3-7 cycles of routine chemotherapy before surgery. Right hemihepatectomy (n=11), left hemihepatectomy (n=2), left trisegmentectomy (n=1), and right trisegmentectomy (n=5) and middle hepatectomy (n=2) were performed. Partial resection of the involved IVC together with the tumor was performed in 5 cases, in which, the IVC wall defect was repaired with a traumatic suture in 4 cases, the IVC wall was end-to-end anatomized in one case. Push-away and dissection of the IVC was performed in 16 cases. No death occurred in this series of patients. The intraoperative hemodynamics was stable in all patients. Patients were observed in the intensive care unit for 3.4±0.3 days. Postoperative complications were in 3 children: in which, one baby had sepsis, fever, jaundice, intra-abdominal abscess, and sub-diaphragmatic abscess with pleural effusion. All the patients were confirmed by pathological examination (fetal type, 13 cases; embryonic type, 4 cases; undifferentiated type, 4 cases). Overall and disease-free 5-year survival rates of the 21 patients with solitary HCC invading inferior vena cava were 18% and 18%, respectively. Conclusion Giant HB attached to the IVC wall in children can be completely removed with a safe operation and can achieve a good prognosis. Careful separation of the liver and IVC is a key point for minimizing the size of the resected IVC and to avoid unnecessary IVC resection.