| Objective: In the treatment of Hepatoblastoma(HB), combined multidisciplinary diagnosis and treatment is becoming a standard treatment for HB currently. Hepatoblastoma Wuhan Protocol is the first national protocol in China for children with HB. We retrospectively analyze the patients with HB in Xinhua Hospital using Wuhan protocol, and assess the feasibility and effectiveness of the protocol.Methods: We retrospective analyzed treatment and long term follow of 47 patients with HB during 2008-2013 in Xinhua hospital, special in demographic characteristics, clinical manifestations, diagnostic methods, Pretext stage, COG stage, chemotherapy treatment, pathology subtype, serum AFP level changes, ?-catenin expression, complications of treatment and treatment effects. All statistical evaluations were done with SPSS version 19.0. Survival curves were estimated according to Kaplan Meier, Chi-square test was estimated according to Fisher exact test.Results: 47 patients with HB(excluding one case who was diagnosed as yolk sac tumor after the surgery) are included in the analysis. Among 46 patients, there are 34 males and 12 females(ratio was 2.83:1), the median age range is 13.75(1.3-126) months. The 6-year overall survival rate and 6-year event-free survival rate were(80.5±12.7) % and(69.9±13.9) % respectively. The percentage of PRETEXT III and IV were 78.3%(36/46). PRETEXT II, III and IV patients event-free survival rate were 100%,(72.2±10.6)% and(33.3±19.2)% respectively(P=0.049). The 6y-EFS rates of COG I, II, III, IV stage patients were(86.6±6.3)%, 100%,(25.0±21.7)% and(18.8±15.8)% respectively(P=0.000). Twenty-five patients with PRETEXT stage III and IV underwent neoadjuvant chemotherapy before surgery, 22 cases( 88%) had complete tumor resection. Patients treated with neoadjuvant chemotherapy had a higher overall survival rate(96.7±3.3)% than with surgery(70.0±14.5%) at initial diagnosis(P=0.009). The fatal group had a good overall survival rate,(88.9±10.5)%(P=0.843). Patients with metastases had a poor prognosis than patients without metastases at diagnosis(6y-OS:(25.0±19.8) % Vs(92.0±4.4) %, P=0.000) Twelve patients performed immunohistochemistry analysis of ?-catenin, seven cases showed positive cytoplasm staining. The overall survival in patients with cytoplasm ?-catenin positive was 100%, higher than that of negative group(80.0±17.9) %. After stratified according to COG group risk stratification criteria, the overall survival rate of very low risk group(1 case) and low risk group(6 cases) were 100 %. The middle risk group(30 cases) overall survival rate was(89.9 ± 5.5)%, and high risk group( 8 cases) overall survival was(25.0 ± 19.8)%, showing a significant statistical difference(P = 0.001).Conclusion:The application of neoadjuvant chemotherapy significantly improved the rate of complete resection and reduced the risk of surgery in HB patients. Combined multidisciplinary treatment should become the standard treatment for patients with HB. The prognostic factors included PRETEXT stage, COG stage and metastasis on set. ?-catenin positive in cytoplasm had not obvious effect on children with HB. Since PRETEXT III and IV stage accounted for higher proportion in whole group, we emphasize the importance of early diagnosis of HB. The patients treated with HB Wuhan protocol had a close overall survival rate and event-free survival rate to the other group in developed countries, which showing that the protocol is feasibility. We should focus on the stratified treatment. Aim to increasing the long-term survival rate for high risk HB patients and reducing complications of treatment, patients with different risk stratification should treat respectively... |
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