Excellent Outcomes of Preoperative Chemotherapy and Surgery for Initially Unresectable Hepatoblastoma in Children / 임상소아혈액종양

ABSTRACT

BACKGROUND: Hepatoblastoma is the most common primary malignant tumor of the liver in children. Complete surgical resection is the treatment of choice for cure. However, only 50% are eligible for resection at diagnosis. Recently combination of preoperative chemotherapy and surgery had led improved resectability and survival rate.METHODS: Between May, 2001 and November, 2010, 9 patients were diagnosed with initially unresectable hepatoblastoma at the department of pediatrics, Yeungnam University Hospital. Medical records were reviewed retrospectively. Initial evaluation included complete blood counts, liver function, serum AFP, cholesterol level and abdominal-CT scan. Preoperative chemotherapy was consisted of cisplatin and doxorubicin every 3-4 weeks. Second-line chemotherapy was cisplatin, vincristine and fluorouracil. The treatment response was analyzed by the Response Evaluation Criteria In Solid Tumors (RECIST) criteria.RESULTS: Among 9 patients, malefemale was 45. Median age at diagnosis was 12 months (4-59 months). The most common presenting symptom was the abdominal mass. Laboratory findings revealed median AFP 216,841 ng/dL (3,535-1,036,404 ng/dL), anemia 4, thrombocytosis 5, elevated AST/ALT 8, hyperbilirubinemia 1 and hypercholesterolemia 5. The median tumor size was 11 cm (8-15 cm). No patient had metastasis. After median 4 (3-5) cycles of preoperative chemotherapy, all patients(100%) showed a partial response and underwent complete surgical resection. Postoperative chemotherapy was given for median 4 (3-5) cycles. The median follow up was 34 months (6-117 months) and all patients are surviving without events.CONCLUSION: Although this study includes limited number of cases, preoperative intensive chemotherapy and surgery for initially unresectable hepatoblastoma in children resulted in excellent outcomes.