| Objective:Hepatoblastoma(HB)is the most common primary liver malignant tumor in childhood.The onset of the disease is insidious and the prognosis varies greatly.At present,there is no unified plan for the risk stratification of HB in the world.The International Childhood Liver Tumour Strategy Group(SI OPEL)proposed a preoperative pretreatment extent of disease(PRETEXT)staging system,emphasizing the impact of tumor involvement and distant metastasis on the prognosis,and based on this classification of HB for standard risk group and high risk group.Children’s Oncology Group(COG)proposed the postoperative COG Evans staging system,emphasizing the impact of postoperative tumor residues on the prognosis,and adopted the PRETEXT staging system to divide HB into four risks:extremely low risk,low risk,intermediate risk and high risk degree.According to the opinions of COG and SIOPEL,Chinese Anti Cancer Association Pediatric Committee formulated"Expert Consensus for Multidisciplinary Management of Hepatoblastoma"(CCCG-HB-2016)in 2016,and put forward the risk stratification suitable for our country.Children’s Hepatic tumors International Collaboration(CHIC)integrated data from multiple research groups,and in 2016 proposed a new international stratification system called the Children’s Hepatic tumors International CollaborationHepatoblastoma Stratification(CHIC-HS).This study intends to retrospectively analyze the clinical data of children with HB admitted to our hospital,summarize the clinical characteristics and survival of HB,explore the risk factors affecting the prognosis of HB.Compare the ability of CCCG-HB-2016 and CHIC-HS to predict the prognosis of children with HB,and provide references for clinical diagnosis and treatment of HB and prognostic judgment.Methods:The clinical data of 83 children with HB treated in the first affiliated Hospital of Zhengzhou University from January 2012 to October 2019 were analyzed retrospectively.All the children were diagnosed as HB by pathology.Summarize the age and clinical manifestations of HB,and analyze the risk factors that affect the prognosis.According to the two classification methods of CCCG-HB-2016 and CHIC-HS,the risk is re-stratified.The survival rate was calculated by Kaplan-Meier survival analysis,and the survival curve was drawn.Log-rank test was used in univariate analysis.The variables with P<0.1 were included in the multivariate COX regression model.Kappa test was used to evaluate the consistency of CCCG-HB-2016 and CHIC-HS risk stratification methods.The area under the curve(AUC)of the receiver operating curve(ROC)was used to evaluate the ability of the two risk stratification methods to predict the prognosis of children.The difference of AUC was tested by MedCalc software Delong test.Results:1.General clinical data:Among 83 children,51(61.4%)were males,32(38.6%)were females,and the ratio of male to female was 1.59:1;the average age of the children was(25.22±24.5)months.The predilection age of HB was under 3 years old,accounting for 77.1%.The most common clinical symptoms were abdominal mass(54.2%),followed by abdominal pain or abdominal distension(19.3%)and unexplained fever(14.5%).The rare clinical symptoms were liver mass during pregnancy(4.8%),acute abdomen caused by tumor rupture(3.6%),anorexia(2.4%)and diarrhea(1.2%).2.Serum AFP levels:64(77.1%)cases were diagnosed with AFP>1000ng/ml for the first time,18(21.7%)cases were diagnosed with AFP of 101-1000ng/ml,and only 1 case(1.2%)was diagnosed with AFP≤100ng/ml.There were 69 patients who had completed 3 courses of chemotherapy after surgery,of which 44(63.8%)patients had AFP dropped to normal level after 3 courses of chemotherapy,and AFP of 25(36.2%)patients did not drop to normal level.3.Tumor growth and invasion:According to PRETEXT staging,8(9.6%)cases were in stage I,43(51.8%)cases in stage Ⅱ,20(24.1%)cases in stage Ⅲ,and 12(14.5%)cases in stage Ⅳ.41(49.4%)cases were located in the right lobe of the liver,10(12.0%)cases were located in the left lobe,and 32(38.6%)cases were located in the left and right liver lobes.65(78.3%)cases had a single tumor,and 18(21.7%)cases had multiple tumors.The tumor diameter ranged from(15-158)mm,and the average diameter was(97.39±28.11)mm.44(53.0%)cases had tumor diameters less than 10 cm,and 39(47.0%)cases had diameters≥10 cm.There were 53(63.9%)cases of epithelial type,18(21.7%)cases of mixed epithelial and mesenchymal type,and 12(14.4%)cases had no clear classification due to less biopsy.Lung metastasis occurred in 14(16.9%)cases,of which 1 case was complicated with bone marrow metastasis.Blood vessels were involved in 13(15.7%)cases.Extrahepatic abdominal disease occurred in 2(2.4%)cases,gallbladder infiltration in 1 case,and metastasis of transverse colonic mucosa and lesser omental cystic tumor in 1 case.Tumor rupture or intraperitoneal hemorrhage occurred in 9(10.8%)cases.VPEFR was positive in 31(37.3%)cases and negative in 52(62.7%)cases.4.Survival analysis showed that the 1-year overall survival rate and event-free survival rate of HB children were(83.7±4.1)%and(78.7±4.6)%,respectively,and the 5-year overall survival rate and event-free survival rate were(78.1±4.7)%and(76.0±4.8)%,respectively.5.Prognostic risk factors:Univariate analysis showed that multifocal tumor,positive VPEFR,distant metastasis,stage Ⅳ of PRETEXT and positive AFP after 3 courses of postoperative chemotherapy were risk factors for prognosis.The results of multivariate COX regression analysis showed that AFP did not turn negative after 3 courses of chemotherapy(HR=9.228,95%CI:1.017～83.692,P=0.048)and stage Ⅳof PRETEXT(HR=6.587,95%CI:1.687～25.723,P=0.007)were independent risk factors affecting the prognosis of children with HB.6.The risk stratification was carried out according to the CCCG-HB-2016 scheme,including 2(2.4%)cases of very low risk,37(44.6%)cases of low risk,15(18.1%)cases of medium risk and 29(34.9%)cases of high risk.According to the CHIC-HS scheme,there were 36(43.3%)cases of very low risk,7(8.4%),cases of low risk 20(24.1%)cases of medium risk and 20(24.1%)cases of high risk.The consistency of the two classification methods is poor,and the Kappa coefficient is 0.225.By ROC test,the results showed that the area under the curve of CCCG-HB-2016 risk stratification method was 0.827.The area under the risk stratification curve of CHIC-HS was 0.876.The ROC curve was tested by DeLong test with MedCalc software,and the difference was not statistically significant.(P=0.14)Conclusions:1.Hepatoblastoma usually occurs in children under 3 years of age.The most common clinical manifestation of hepatoblastoma is abdominal mass,as well as non-specific symptoms such as abdominal pain,abdominal distension,fever,anorexia and acute abdomen caused by tumor rupture.2.The prognosis of the children whose AFP does not decrease to normal level after 3 courses of postoperative chemotherapy is poor,so the changes of AFP should be monitored in the course of treatment to guide the treatment.3.The PRETEXT staging is of great value to the prognosis.At the first diagnosis,relevant imaging examinations such as abdominal ultrasound,abdominal CT and chest CT should be perfected,the PRETEXT staging should be clarified,and the treatment plan should be formulated through multidisciplinary consultation.4.The consistency of CCCG-HB-2016 risk stratification method and CHIC-HS risk stratification method is poor,but both of them can better predict the prognosis. |
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