Risk factors for clinically relevant postoperative pancreatic fistula of pancreatic tumor in children / 中华肝胆外科杂志

Objective:To analyze the influencing factors of clinically relevant postoperative pancreatic fistula (CR-POPF) in children with pancreatic tumors after surgery.Methods:The clinical data of 123 children undergoing surgery for pancreatic tumor in Beijing Children's Hospital, Capital Medical University from January 2007 to March 2020 were retrospectively analyzed, including 39 males and 84 females, with a median age of 9.8 years (6.7 to 11.8). Patients without pancreatic fistula and with biochemical leakage were included in control group ( n=95), while patients with grade B and C pancreatic fistula were divided into CR-POPF group ( n=28). The independent influencing factors of CR-POPF were analyzed by univariate and multivariate logistic regression. Results:Among 123 children, 28 cases (22.8%) developed CR-POPF, including 24 cases (85.7%, 24/28) of grade B pancreatic fistula and 4 cases (14.3%, 4/28) of grade C pancreatic fistula. There were significant differences between CR-POPF and control groups in the age > 8 years and 4 months, tumor location, operation time >390 min and procedures (all P<0.05). Multivariate logistic regression analysis showed an increased risk of CR-POPF in children aged > 8 years and 4 months ( OR=8.226, 95% CI: 1.813-37.333, P=0.006) and undergoing duodenum-preserving pancreatic head resection (DPPHR) ( OR=3.353, 95% CI: 1.282-8.767, P=0.014). Conclusion:Age>8 years and 4 months and DPPHR are independent risk factors for CR-POPF in children with pancreatic tumors after surgery.

Clinical characteristics and prognosis of neuroblastoma in the pelvic and sacral regions / 中华实用儿科临床杂志

Objective:To summarize the clinical characteristics, treatment response and long-term postoperative complications in children with neuroblastoma (NB) in the pelvic and sacral regions as the primary site.Methods:The clinical characteristics of 16 NB children (8 males and 8 females) with primary pelvic and sacral admitted to the Department of Hematology Oncology Center in Beijing Children′s Hospital, Capital Medical University from March 2007 to June 2019 were analyzed retrospectively with respect to the age at first diagnosis, primary tumor site, tumor size, clinical stage, risk grouping, and other clinical characteristics.The clinical characteristics of the patients who were followed up for regular treatment were analyzed, and the postoperative complications of the patients were summarized, and the Kaplan-Meier method was used for survival analysis.Results:The median age at diagnosis of these 16 children was 23.0 months (5.7-102.0 months), of which 6 cases (37.5%) were younger than 12 months old.All these children received chemotherapy, with a median of 6 (1-8) courses of chemotherapy.Fifteen children received surgical resection of the pelvic tumor, with complete resection in 12 cases (80%). The surgical approach was mainly transabdominal (86.7%, 13/16 cases). The median follow-up time of these children was 33.5 (8-136) months.The patella was absent in 3 patients (18.8%) after the operation, and no permanent neurological damage occurred in all patients.Five-year overall survival (OS) rate was 100%.Conclusions:A single-center summary showed a high survival rate for NB patients in the pelvic and sacral regions.Complete tumor resections combined with chemotherapy could be effective measures and rare cases occurred permanent postoperative neurological complications.

Analysis of early death in children with neuroblastoma / 中华实用儿科临床杂志

Objective:To summarize the causes of death and severe complication in the early diagnosis of children with neuroblastoma (NB), and to analyze the relative factors of early death of children with NB, so as to raise awareness and reduce early mortality by early detection and early intervention.Methods:Patients with newly diagnosed NB in the Hematology Oncology Center of Beijing Children′s Hospital from April 2007 to December 2017 were included consecutively, and those died within 1 month after diagnosis were retrospectively analyzed.The general data of patients, immediate causes of death, complications, time elapsed between death and diagnosis, whether to receive chemotherapy and other information were collected.Results:A total of 654 cases were included for diagnosis, treatment and follow-up, 31 cases of which died in early stage, accounting for 4.7% of the total.The major complication were pulmonary infection in 18 cases (58.1%) and bone marrow suppression after chemotherapy in 17 cases (54.8%), tumor rupture hemorrhage in 16 cases (51.6%), multiple organ failure in 8 cases (25.8%). Risk factor analysis of the 31 early death cases with NB was conducted.Single factor analysis: there were statistical differences between early death group and non-early death group in risk grouping ( P=0.006 6), bone marrow invasion ( P=0.020 7), site of primary tumor ( P=0.016 7), age ( P=0.003 3), lactate dehydrogenase (LDH) level ( P<0.000 1), neuron-specific enolase (NSE) level ( P<0.000 1), serum ferritin level ( P=0.016 0), D dimer level ( P<0.000 1), fibrinogen level ( P=0.002 7), diameter of tumor ( P<0.000 1), hemoglobin ( P<0.000 1), platelet level ( P<0.000 1), serum albumin level ( P<0.000 1). Multiple-factor analysis: age younger than 30 months, OR=2.824 (95% CI: 1.084-7.359), LDH level greater than 1 004 IU/L, OR=6.991 (95% CI: 2.135-22.887), albumin level less than 36 g/L, OR= 65.237 (95% CI: 2.024-13.545), hemoglobin level less than 92 g/L, OR=5.358 (95% CI: 2.024-13.545), platelet level less than 192×10 9/L, OR=3.554 (95% CI: 1.267-9.965). Conclusions:Strengthening vital signs detection after admission, identifying severe life-threatening complications such as rupture of tumors as early as possible, implementing symptomatic interventions such as appropriate sedation and active transfusion of blood products as early as possible after invasive operation, and transferring to intensive care unit for respiratory support when necessary are important means to avoid early death.

Clinical characteristics and long-term survival quality in single-center children with low and intermediate-risk neuroblastoma / 中华实用儿科临床杂志

Objective:To summarize the clinical characteristics of single-center children with low and intermediate-risk neuroblastoma (NB), report the long-term follow-up results of the growth and survival quality, and provide a basis for further clinical research.Methods:Clinical characteristics, including the sex, age, stage, risk of disease, and metastatic site of 370 newly treated children with low and intermediate-risk NB admitted to Hematology Oncology Center, Beijing Children′s Hospital from March 2007 to June 2019 were retrospectively analyzed.Kaplan-Meier method was used for survival analysis.WHO Anthro Plus was used for calculating Z score.Results:A total of 370 eligible children with low and intermediate-risk NB were included, with the mean age at onset of 16.8 months (1-191 months). Among them, 148 cases (40%) were younger than 12 months old.Mediastinal region was the most common primary site of NB (47.8%, 177 cases), followed by retroperitoneum/adrenal gland (41.4%, 153 cases). The median follow-up time of 370 patients was 31 months (0.3-157.0 months), the 5-years event free survival (EFS) and 5-year overall survival (OS) were 86.2% and 96.9%, respectively.Thirty-seven cases had growth and deve-lopment problems, of which 22 cases had stunted growth, 6 cases had low body mass, 9 cases had wasting, and 7.3%(27/370 cases) had scoliosis.5.5% of them had heart damage and 5.0%(18/357 cases) had kidney damage, involving 12 cases related to the primary tumor and 6 cases were surgically related.30.2%(95/315 cases) of them had hair changed after chemotherapy, and curly hair was the most common change.Compared with before treatment, 14.9% of the children had a personality change, with an impatient being the most common.Conclusions:The 5-year overall survival rate of the single-center large sample of low and intermediate-risk NB was high, mediastinal was the most common primary site of tumor, and the long-term quality of life is good, but there were still treatment-related side effects, and further clinical monitoring and long-term follow-up were needed.

Clinicopathological characteristics and treatment of hepatoblastoma with macrotrabecular structures / 中华肝胆外科杂志

Objective:To analyze the clinicopathological characteristics and treatment strategies of hepatoblastoma with macrotrabecular structures.Methods:To retrospectively analyze the data of children with hepatoblastoma treated in the Department of Oncology at Beijing Children's Hospital affiliated to Capital Medical University amd Baoding Children's Hospital from January 1, 2011 to December 31, 2019. The study structure consisted of collecting clinical data and formulating treatment plan, including clinical stage, alpha-fetoprotein (AFP), chemotherapy plan, surgical margin, pathological classification and follow-up data to study the long-term prognosis of these patients.Results:Among 17 patients, 13 males and 4 females, age 5 to 134 months; 5 patients had the macrotrabecular type, 10 patients had the epithelial type with macrotrabecular, 2 patients had the mixed epithelial and mesenchymal with macrotrabecular type. For the PRETEXT staging, 1 patient had stage I, 2 patients had stage II, 14 patients had stage III and IV. A total of 15 patients received preoperative chemotherapy, and 6 achieved partial response. Among 17 patients, 10 had negative resection margins. The AFP of 7 patients was normal after chemotherapy, and 10 patients relapsed after surgery. The 2-year event-free survival was 26.18%.Conclusions:Patients with hepatoblastoma containing giant trabecular components are very rare. Among them, the recurrence rate of patients with giant trabecular and epithelial and giant trabecular components was high. Preoperative neoadjuvant chemotherapy (vincristine + irinotecan), transcatheter arterial chemoembolization and liver transplantation are treatment options for this type of hepatoblastoma.

Clinical features and survival analysis of neuroblastoma with cervical lymph node metastasis in children: su-mmary of 10 years in single center / 中华实用儿科临床杂志

Objective:To improve the awareness of neuroblastoma (NB) with cervical lymph node metastasis in children patients by summarizing the clinical features of such patients and analyzing their survival situation.Methods:The research analyzed the medical records of 225 patients with cervical lymph node metastasis of NB from April 1, 2007 to May 31, 2017 in Beijing Children′s Hospital, Capital Medical University.The treatment were divided into 2 phases according to treatment time (one from April 1, 2007 to December 31, 2011 and the other from January 1, 2012 to May 31, 2017). The survival situation and treatment effect in each phase were analyzed.The following up time ended at Jan 1, 2018.Results:(1)Clinical features: total 225 cases admitted in the research with about 37.3% of all the NB patients.One hundred girls(44.4%) and 125 boys(55.6%). The age from 2 months to 147 months, the me-dian age of patients was 37 months.The age under 18 months were 27 cases(12.1%). The majority primary site of tumor was located in retroperitoneal(174 cases, 77.3%) and mediastinum(48 cases, 21.3%). There were 33 cases which metastasic site confined to cervical lymph node(4N stage, 14.7%); 139 cases (62.1%) with bone marrow metastasis, 159 cases (71.0%) with bone metastasis.The number of patients in high-risk group was 208 cases(91.2%), while the low-risk and the medium-risk group was 17 cases(7.5%). (2)Treatment effect and survival analysis: a total of 200 cases received systemic treatment and followed-up in our hospital.The follow up time from 2 months to 123 months, the median follow up time was 23 months.The 3 year overall survival (OS) rate of all the patients was 56.4%, the 3 year event free survival (EFS) rate was 52.0%.While the 5-year OS was 46.5%, and the 5-year EFS was 39.7%.According to the first phase of treatment(April 1, 2007 to December 31, 2011), the 3-year EFS was 49.6%, and the 3-year OS was 54.4%, the 5-year EFS was 39.5%, and the 5-year OS was 44.7%.According to the second phase of treatment(January 1, 2012 to May 31, 2017), the 3-year EFS was 58.1%, and the 3-year OS was 57.3%.The 5-year EFS was 48.7%, and the 5-year OS was 46.8%.About the 4N patients, the 3-year EFS was 71.2%, and the 3-year OS was 82.2%.The 5-year EFS was 61.5%, and the 5-year OS was 76.7%.Conclusions:Cervical lymph node is one of the most common distant metastasis of NB.More than half of the patient with bone or bone marrow metastasis.The prognosis of patients′ metastatic disease limited to cervical lymph nodes(4N stage), but still worse than the foreigner haver reported.The probable reason is the proportion of high-risk patients in the center is higher than the foreign research.

A summary of single center diagnosis and treatment experience of extracranial malignant germ cell tumor in children / 中华实用儿科临床杂志

Objective:To summarize the clinical features, therapeutic effects and prognostic factors of children with extracranial malignant germ cell tumor (MGCT), and further to improve the recognition of this disease.Methods:The clinical information and therapeutic effects of children patients diagnosed with MGCT and treated in Hematology-Oncology Center in Beijing Children′s Hospital, Capital Medical University from January 2008 to December 2018 were analyzed retrospectively, and the risk factors affecting the prognosis were discussed.Results:A total of 35 patients were collected in this study, including 19 girls and 16 boys, with a median age of 71 months.Forty-two point four percent (14/33 cases) of them were <3 year-old, and 39.3% (13/33 cases) of them were >6 year-old.The primary tumors were located at the gonad in 9 cases (6 cases in the ovary and 3 cases in the testis), and extragonadal sites in 26 cases (8 cases in the pelvic cavity, 8 cases in the mediastinum, and 10 cases in the sacrococcygeal region). Five cases were in stage Ⅱ, 16 cases in stage Ⅲ, and 14 cases in stage Ⅳ.Eleven cases were at intermediate risk, and 24 cases were at high risk.All children were followed up for (25.43±4.82) months, and given standard treatment of surgery plus chemotherapy.The 2-year overall survival (OS) rate and event-free survival (EFS) rate were 87.4% and 67.1%, respectively.The 2-year OS rate and EFS rate in patients at stage Ⅱ and Ⅲ (93.8% and 80.4%, respectively) were significantly higher than those at stage Ⅳ (66.7% and 45.1%, respectively) ( χ2=0.649, 0.739; P=0.021, 0.037). The 2-year EFS rate in patients with and without distant metastasis at the early stage of the disease was 43.8% and 86.9%, respectively, and the differences were significant ( χ2=0.694, P=0.028). The complete remission (CR) rates of intermediate-risk and high-risk children with tumor completely resected at the early stage of the disease were 100.0% (8/8 cases) and 71.4% (5/7 cases), respectively.The CR rate of patients who received 2-4 cycles of chemotherapy and had complete tumor resection after diagnosis by puncture was 72.7% (8/11 cases), and 40.0% (2/5 cases) of the children with incomplete resection got tumor progression.For eight patients with events, their alpha-fetoprotein (AFP) levels decreased slowly at the early stage of the disease and increased significantly as the disease progressed. Conclusions:The onset-age of MGCT is mainly <3 year-old and >6 year-old.The stan-dard treatment that combines surgery and chemotherapy can achieve a good overall prognosis.Incomplete surgical resection and a slow decline of AFP during treatment contribute to adverse prognosis.The outcomes of patients with tumor progression can be improved by increasing chemotherapy courses, secondary surgery and autologous stem cell transplantation.

Clinical and prognostic analysis of single-center multidisciplinary treatment for rhabdomyosarcoma in children / 中华儿科杂志

Objective@#To summarize the clinical characteristics, treatment response and prognostic factors of rhabdomyosarcoma (RMS) in children.@*Methods@#The clinical characteristics such as age at diagnosis, primary tumor site, tumor size, pathological type, clinical stage, and risk grouping of 213 RMS patients (140 males and 73 females) treated in Hematology Oncology Center of Beijing Children′s Hospital, Capital Medical University, from May 2006 to June 2018 were analyzed retrospectively. The clinical characteristics, overall survival (OS), event free survival (EFS) and prognostic factors of children treated with the Beijing Children′s Hospital-Rhabdomyosarcoma (BCH-RMS) regimen were analyzed. Survival data were analyzed by Kaplan-Meier survival analysis, and single factor analysis was performed by Log-Rank test.@*Results@#The diagnostic age of 213 cases was 48.0 months (ranged 3.0-187.5 months), of which 136 cases (63.8%) were younger than 10 years old. The head and neck region was the most common primary site of tumor (30%, 64 cases), followed by the genitourinary tract (26.8%, 57 cases). Among pathological subtypes, embryonal RMS accounted for 71.4% (152 cases), while alveolar RMS and anaplastic RMS accounted for only 26.8% (57 cases) and 1.9% (4 cases), respectively. According to the Intergroup Rhabdomyosarcoma Study Group (IRS), IRS-Ⅲ and Ⅳ accounted for 85.0% (181 cases) of all RMS patients. In all patients, 9.4% (20 cases) patients were divided in to low-risk group, 52.1% (111 cases) patients in to intermediate -risk group, 25.8% (55 cases) patients in to high-risk group, and 12.7% (27 cases) patients in to the central nervous system invasion group, respectively. All patients with RMS received chemotherapy. The cycles of chemotherapy were 13.5 (ranged 5.0-18.0) for patients without event occurrence, while 14.2 (ranged 3.0-30.0) for patients with event occurrence. Among the 213 patients, 200 patients had surgical operation, of whom 103 patients underwent surgery before chemotherapy and 97 patients at the end of chemotherapy, 21 patients had secondary surgical resection. Radiotherapy was performed in 114 patients. The follow-up time was 23.0 months (ranged 0.5-151.0 months) . There were 98 patients with relapsed or progressed disease and 67 patients with death. The median time to progression was 10 months, of which 67 (68.4%) relapse occurred within 1 year and no recurrence occurred after follow-up for more than 5 years. The 3-year EFS and 5-year EFS were (52±4) % and (48±4) %, while the 3-year OS and 5-year OS were (65±4) % and (64±4) % by survival analysis. The 5-year OS of the low-risk, intermediate-risk, the high-risk were 100%, (74±5) %, (48±8) %, and the 2-year OS of the central nervous system invasion group was (36±11) % (χ2=33.52, P<0.01). The 5-year EFS of the low-risk, intermediate-risk, the high-risk were (93±6) %, (51±5) %, (36±7) % and the 2-year EFS of the central nervous system invasion group was (31±10) % (χ2=24.73, P<0.01) . Survival factor analysis suggested that the OS of children was correlated with age(χ2=4.16, P=0.038), tumor TNM stage (χ2=22.02, P=0.001), IRS group (χ2=4.49, P<0.01) and the risk group (χ2=33.52, P<0.01).@*Conclusions@#This study showed that the median age of newly diagnosed RMS patients was 4 years. The head and neck and the genitourinary tract were the most common primary origin of RMS. The OS was low in single-center RMS children. The median time to recurrence was 10 months, and recurrence was rare 3 years later.

Genomic DNA detection frequently-used chemotherapeutic drugs and their adverse drug reactions in children with rhabdomyosarcoma / 中华实用儿科临床杂志

Objective To analyze and summarize the results of genomic DNA test findings of chemotherapeutic drugs commonly used in pediatric rhabdomyosarcoma (RMS) in children,and to analyze the relationship between adverse reactions to chemotherapy toxicity and genomic DNA polymorphisms,so as to provide evidence for guiding treatment.Methods Retrospective analysis was conducted in RMS children admitted at Hematology Oncology Center,Beijing Children's Hospital,Capital Medical University from January 2017 to June 2018.The criteria for enrollment were definite diagnosis of RMS,regular treatment and follow-up at Hematology Oncology Center,Beijing Children's Hospital,Capital Medical University,and detection of peripheral blood DNA fluorescence hybridization sequence for several commonly chemotherapy drugs.The toxicity of chemotherapeutic drugs was detected based on the National Cancer Institute routine toxicity criteria (NCI-CTCAE version 4.0).Summary and analysis indicators included primary and metastatic site,size,international RMS clinical stage (TNM-UICC),Intergroup Rhabdomyosarcoma Study (IRS) Clinical Grouping Classification,risk grouping,pathological type,changes in major organ functions,as well as processes of surgery,chemotherapy and radiotherapy,and the association between toxicity and DNA polymorphism of drug genes was analyzed.SPSS 22.0 software was used for x2 test.Results A total of 32 children were enrolled,and 20 cases were male and 12 cases were female,their median age was 50 months (15-120 months).The primary tumor of 9 cases were sited in the chest,abdomen and basin,8 cases in the head and neck (non-meningeal),7 cases in bladder prostate,3 cases in limbs,2 cases in the meningeal area,1 case in urogenital tract (non-bladder prostate),2 cases in other parts.Seventeen cases were embryonic type and 15 cases were alveolar type.Five cases were TNM-Ⅰ stage,5 cases were TNM-Ⅱ stage,10 cases TNM-Ⅲ stage,12 cases were TNM-Ⅳ stage,21 cases were IRS-Ⅲ,11 cases were IRS-Ⅳ.Twenty-two cases were moderate-risk (MR),10 cases were high-risk (HR).Twenty-two cases were detected UGT1A1 * 6 gene,18 cases in GG type,13 cases in GA type,and 1 case in AA type.ABCB1 gene monitoring was performed in 27 children,14 cases of CT type and 13 cases of TT type;29 cases were detected GSTP1 gene,7 cases of GA type and 2 cases of GG type,19 cases of AA type,1 case of AG type;30 cases were detected CYP3A5 gene,2 cases of GA type,13 cases of GG type,AG 15 cases.All patients were treated according to the BCH-RMS-2007 protocol using VAC (Vincristine,Doxorubicin,and Cyclophosphamide) as the basis for chemotherapy.From 2017,VAC and Ⅵ regimen (Vincristine,Irinotecan) were defined as the standard of backbone chemotherapeutic regimen for MR.Nine cases underwent surgery before chemotherapy and 10 cases had surgery after chemotherapy,among them,5 cases underwent twice operation.Local radiotherapy was performed on the 12th week of chemotherapy,and the central nervous system involvement cases started in the first week.Hematological toxicity was mainly caused by neutropenia,with 2 cases of grade 3 and 30 cases of grade 4.Liver function damage of grade 2 was 6 cases,grade 3 was 3 cases.Four patients with grade 1 diarrhea,3 patients with grade 2,5 patients with grade 3,3 patients with grade 4.There was significant difference between the severity of diarrhea and UGT1A1 * 6 genotype polymorphism (P ＜ 0.05).Conclusions Chemotherapy for RMS patients is highly safety.If the genomic DNA test of chemotherapy drugs show a slow metabolism type,the dose of chemotherapy should be reduced,and the toxicity of chemotherapy drugs should be monitored dynamically.

Genomic DNA detection frequently-used chemotherapeutic drugs and their adverse drug reactions in children with rhabdomyosarcoma / 中华实用儿科临床杂志

Objective@#To analyze and summarize the results of genomic DNA test findings of chemotherapeutic drugs commonly used in pediatric rhabdomyosarcoma (RMS) in children, and to analyze the relationship between adverse reactions to chemotherapy toxicity and genomic DNA polymorphisms, so as to provide evidence for guiding treatment.@*Methods@#Retrospective analysis was conducted in RMS children admitted at Hematology Oncology Center, Beijing Children′s Hospital, Capital Medical University from January 2017 to June 2018.The criteria for enrollment were definite diagnosis of RMS, regular treatment and follow-up at Hematology Oncology Center, Beijing Children′s Hospital, Capital Medical University, and detection of peripheral blood DNA fluorescence hybridization sequence for several commonly chemotherapy drugs.The toxicity of chemotherapeutic drugs was detected based on the National Cancer Institute routine toxicity criteria (NCI-CTCAE version 4.0). Summary and analysis indicators included primary and metastatic site, size, international RMS clinical stage (TNM-UICC), Intergroup Rhabdomyosarcoma Study(IRS) Clinical Grouping Classification, risk grouping, pathological type, changes in major organ functions, as well as processes of surgery, chemotherapy and radiotherapy, and the association between toxicity and DNA polymorphism of drug genes was analyzed.SPSS 22.0 software was used for χ2 test.@*Results@#A total of 32 children were enrolled, and 20 cases were male and 12 cases were female, their median age was 50 months (15-120 months). The primary tumor of 9 cases were sited in the chest, abdomen and basin, 8 cases in the head and neck (non-meningeal), 7 cases in bladder prostate, 3 cases in limbs, 2 cases in the meningeal area, 1 case in urogenital tract (non-bladder prostate), 2 cases in other parts.Seventeen cases were embryonic type and 15 cases were alveolar type.Five cases were TNM-Ⅰ stage, 5 cases were TNM-Ⅱ stage, 10 cases TNM -Ⅲ stage, 12 cases were TNM-Ⅳ stage, 21 cases were IRS-Ⅲ, 11 cases were IRS-Ⅳ.Twenty-two cases were moderate-risk (MR), 10 cases were high-risk (HR). Twenty-two cases were detected UGT1A1*6 gene, 18 cases in GG type, 13 cases in GA type, and 1 case in AA type.ABCB1 gene monitoring was performed in 27 children, 14 cases of CT type and 13 cases of TT type; 29 cases were detected GSTP1 gene, 7 cases of GA type and 2 cases of GG type, 19 cases of AA type, 1 case of AG type; 30 cases were detected CYP3A5 gene, 2 cases of GA type, 13 cases of GG type, AG 15 cases.All patients were treated according to the BCH-RMS-2007 protocol using VAC (Vincristine, Doxorubicin, and Cyclophosphamide) as the basis for chemotherapy.From 2017, VAC and VI regimen (Vincristine, Irinotecan) were defined as the standard of backbone chemotherapeutic regimen for MR.Nine cases underwent surgery before chemotherapy and 10 cases had surgery after chemotherapy, among them, 5 cases underwent twice operation.Local radiotherapy was performed on the 12th week of chemotherapy, and the central nervous system involvement cases started in the first week.Hematological toxicity was mainly caused by neutropenia, with 2 cases of grade 3 and 30 cases of grade 4.Liver function damage of grade 2 was 6 cases, grade 3 was 3 cases.Four patients with grade 1 diarrhea, 3 patients with grade 2, 5 patients with grade 3, 3 patients with grade 4.There was significant diffe-rence between the severity of diarrhea and UGT1A1*6 genotype polymorphism(P<0.05).@*Conclusions@#Chemothe-rapy for RMS patients is highly safety.If the genomic DNA test of chemotherapy drugs show a slow metabolism type, the dose of chemotherapy should be reduced, and the toxicity of chemotherapy drugs should be monitored dynamically.

Short-term complications and long-term outcomes of spleen-preserving distal pancreatectomy in pediatric pancreatic tumors / 中华普通外科杂志

Objective To analyze the short-term complications and long-term outcomes of spleenpreserving distal pancreatectomy (SPDP) in pediatric pancreatic tumors.Methods A retrospective analysis was conducted on 43 patients with tumors of the body and tail of the pancreas in Beijing Children's Hospital from Jan 2007 to Jan 2018.Results There were 17 boys (39.53％) and26girls (60.47％),with a median age of 123 (80,141) months.The median maximum diameter of primary tumor was 7.60 cm.Diagnoses included solid pseudopapillary tumor (n =28),pancreatoblastoma (n =10),neuroendocrine tumor (n =4),and pancreatic cyst (n =1).Two cases (4.65％) received tumor enucleation,4 cases (9.30％) did distal pancreatectomy plus splenectomy,and 37 cases (86.05％) did SPDP.16 cases (37.21％) had short-term complications,including pancreatic fistula (n =13),delayed gastric emptying (n =3),abdominal infection (n =7) and postoperative bleeding (n =2).After a median follow-up of 46 (23,71) months,38 cases (88.37％) were disease-free;two cases (4.65％) with tumor recurrence;one case of pancreatoblastoma died of tumor recurrence.Two cases lost to follow-up.Three patients had long-term complications,including chronic fatty diarrhea (n =2) and hypoglycemia (n =1).Three patients underwent second operation for recurrent tumor (one pancreatoblastoma and two solid pseudopapillary tumor).Conclusions SPDP is safe and effective in the treatment of tumors of the body and tail of the pancreas in children.

Predictive value of age and serum neuron enolase analysis in the pathological prognosis of histopathology sub-group in intermediate and high-risk neuroblastoma / 中华实用儿科临床杂志

Objective To improve substantially the prediction accuracy of histopathology subgroup through combined analysis of clinical and biological features,and accordingly build a decision tree to predict the histopathology subgroup in intermediate and high - risk neuroblastoma. Methods A total of 62 intermediate and high - risk neuro-blastoma patients were included retrospectively in this study,who received chemotherapy and surgery at Beijing Chil-dren's Hospital (BCH),Capital Medical University between January 2015 and January 2017. The fin-dings of urinary vanillylmandelic acid (VMA),and homovanillic acid (HVA),serum neuron - specific enolase (NSE),lactate dehy-drogenase (LDH)and ferritin,ultrasound,CT,MRI,positron emission tomography - computed tomography (PET -CT),bone marrow aspiration and biopsy,MYCN gene,and histopathology were collected and analyzed retrospectively. Statistical analysis was performed by using SAS 9. 4. Univariate and multivariate Logistic regression analysis were con-ducted to select potentially useful characteristics for prediction. Based on the results of Logistic regression analysis,a classification tree was developed to predict histopathology subgroup. Results To identify the characteristics related to histopathology,tumor markers and six clinico - pathologic factors were evaluated by univariate analysis. The results showed that unfavorable histopathology(UH)was more frequently associated with bone marrow metastasis,older age,as well as higher serum NSE,ferritin and LDH levels. The result of multivariate analysis showed that age and NSE were significant independent predictors of histopathology. The adjusted odds ratio(OR)of NSE and age was 33. 2 and 13. 0, respectively. The area under the receiver - operating - characteristic (AUC)of the prediction mo-del was 0. 889. The sensitivity and specificity were 91. 90％ and 76. 00％,respectively. Furthermore,to provide a visua-lization of the sig-nificant predictors found by Logistic regression analysis,a decision tree was developed for predicating of histopathology. Conclusions Age and NSE are significant independent predictors of histopathology subgroup. The decision tree based on age and NSE can help to predict the histopathology subgroup in intermediate and high - risk neuroblastoma effective-ly.

Research advances in molecular biological mechanisms of neuroblastoma / 中华实用儿科临床杂志

Neuroblastoma(NB)is the most common solid extracranial tumor of children,which is obviously heterogenous in the positions,histopathological features,and biological characteristics. One of the most intractable issues in the current clinical treatment is the poor prognosis in high - risk and advanced stage of NB. In recent years,numerous studies have been conducted on molecular biological mechanisms of NB. Researches demonstrate that tumorigenesis is related to various factors,including chromosome,oncogene,and antioncogene abnormalities;carcinogenesis related genes (involving cell proliferation,apoptosis,angiogenesis,chemoresistance and intracellular signal transduction system)ab-normalities. Some specific information(such as chromosome 1p status,MYCN expression,and DNA ploidy)has become the important basis for judging prognosis and grouping the patients by risks. Meanwhile,there are still many discovered NB - related molecular biological mechanisms which are not yet included in the risk stratification system. Further development of targeted drugs for those molecules may be of great significance in increasing the survival rate of patients and improving the prognosis.

Clinical characteristics and gene mutations analysis of 56 patients with congenital hyperinsulinism / 中华实用儿科临床杂志

Objective To analyze the clinical characteristics and gene mutations of 56 patients with congenital hyperinsulinism(CHI)and to provide a theoretical basis for clinical diagnosis and treatment of CHI.Methods Fifty-six children who were diagnosed as CHI between February 2002 and January 2016 in Beijing Children's Hospital Affiliated to Capital Medical University were selected as research subjects.A retrospective study was done about the clinical data and the treatment procedures of the 56 patients,such as perinatal conditions,clinical manifestations,laboratory data,treatments,prognosis and so on.Polymerase chain reaction(PCR)-DNA technology or next-generation sequencing technology was used to analyze the CHI relevant genes of the 56 patients.Results Thirty of the 56 patients carried CHI gene mutation.(1)Twenty-three of 56 patients(41.0％)carried ABCC8/KCNJ11 gene mutations:4 of 23 patients carried complex heterozygous mutation,1 of 23 patients carried both ABCC8 and KCNJ11 gene mutation,1 of 23 patients carried maternally inherited ABCC8 gene mutation,12 of 23 patients carried paternally inherited ABCC8 gene mutation,1 of 23 patients carried paternally inherited KCNJ11 gene mutation,3 of 23 patients carried de novo ABCC8 gene mutation,1 of 23 patients had unknown genetic way,19 of 23 patients were treated with Diazoxide,2 of 19 patients were responsive to Diazoxide,7 of 19 patients were unresponsive to Diazoxide and 10 of 19 patients were uncertain to Diazoxide.(2)Five of 56 patients(8.9％)carried GLUD1 gene mutation,4 of 5 patients were treated with Diazoxide and they were all responsive to Diazoxide.(3)One of 56 patients(1.7％)carried de novo GCK gene mutation,responsive to Diazoxide treatment.(4)One of 56 patients(1.7％)carried maternally inherited SLC16A1 gene mutation,responsive to Diazo-xide treatment.Conclusions The ABCC8 gene and GLUD1 gene mutation are the main causative genes of CHI.The GCK gene and SLC16A1 gene mutation are in the minority.Most ABCC8 gene and KCNJ11 gene mutation are unresponsive to Diazoxide treatment.

Clinical analysis of 194 patients with congenital hyperinsulinism / 中华实用儿科临床杂志

Objective To summarize the clinical data of 194 patients with congenital hyperinsulinism(CHI),in order to provide the theoretical basis for the diagnosis,treatment and prognosis of CHI.Methods One hundred and ninety-four patients with CHI hospitalized in Beijing Children's Hospital Affiliated to Capital Medical University from February 2002 to January 2016 were recruited.Clinical data of 194 patients were collected and clinical characteristics and treatment were retrospectively analyzed.Results One hundred and forty-four cases in 194 patients were treated with Diazoxide,among whom,88 cases were responsive to Diazoxide,23 cases were unresponsive to Diazoxide,and 33 cases were uncertain to Diazoxide.Twenty-six cases in 194 cases were treated with Octreotide,among whom,16 cases were responsive to Octreotide,5 cases were unresponsive to Octreotide,and 5 cases were uncertain to Octreotide.Eleven cases in 194 cases had the 18 Fluoride-L-dihydroxyphenylalanine positron emission computerized tomography scan (18-F-L-DOPA-PET scan),among whom,7 cases were identified as focal lesion and 4 cases were identified as diffuse lesion.Twelve cases in 194 cases were treated with pancreatectomy,among whom,4 cases achieved normal blood glucose levels,4 cases still suffered from persistent hypoglycemia,3 cases had type 1 diabetes mellitus,and 1 case dropped out after the surgery.Forty-five cases in 194 cases had spontaneous remission and the remission time varied from 1 month to 8 years.Conclusions (1) Diazoxide is the first line drug of CHI.The older age onset is,the higher possibility of responsive to Diazoxide shows.(2)Octreotide is used as the second line drug.(3)18-F-L-DOPA-PET scan is the first choice to identify the location of CHI and it is useful in distinguishing between focal and diffuse forms.(4) The outcome of CHI patients after pancreatectomy is very different.A proper surgical method should be selected before the surgery.The blood glucose should be detected after surgery for a long time.(5) Some patients have the tendency of spontaneous remission.

Relationship between serum alpha-fetoprotein level and clinical features of hepatoblastoma in children / 中华普通外科杂志

Objective To analyze the relationship between the clinical features of hepatoblastoma and serum alpha-fetoprotein level.Methods We did a retrospective study in 74 cases into the relationship of clinical stages,treatment,prognosis,and AFP value in hepatoblastoma.Results In Stage Ⅰ and Ⅱ patients,the average AFP was (36 333 ± 13 782) ng/ml and in Stage Ⅲ and Ⅳ,the average AFP was (78 346 ± 27 956) ng/ml,P ＜ 0.05.68 patients received preoperative chemotherapy with alpha-fetoprotein determinations before and after chemotherapy respectively.In 44 cases AFP decreased ＞ 50％ after chemotherapy,and in 20 cases AFP declined ＞90％,while in 13 cases AFP increased after chemotherapy.15 cases had serum AFP measured on the first postoperative day,among them 10 cases had a ＞ 50％ AFP decline,3 cases had a ＜ 50％ AFP decline,while in 2 cases AFP value increased.52 cases were postoperatively followed-up,with overall 3 year-survival rate of 86.5％ ; 7 cases recurred,4 died.The average preop AFP level in 7 recurred cases was (27 060 ± 3 569) ng/ml,while the average preop AFP level in those of 3-year recurrence-free was (29 865 ± 5 867) ng/ml,P ＞ 0.05.Only 57％ tumor recurred cases had back to normal AFP level within one month postop,while 89％ long term survivals reported normal range of AFP during that period,P ＜ 0.05.Conclusions Serum AFP level in patients with hepatoblastoma is related to the course of disease.It can be used to estimate the effect of clinical chemotherapy.The speed of alpha-fetoprotein decline postop can be used as an indicator of prognosis.The postoperative normal AFP level within 4 weeks predicts a favorable prognosis.

The diagnosis and surgical treatment of malignant gastrointestinal tumors in children / 中华消化杂志

Objective To investigate the essential points of the diagnosis and the surgical treatment of malignant gastrointestinal tumors in children.Methods Clinical data involved the sites and pathological types of the tumors,clinical manifestation,effective treatment and prognosis were retrospectively analyzed in 29 consecutive patients reffered to Beijing Children's Hospital between January 1996 and May 2008.Results Of 29 patients,26 were males and 3 were females(aged from 1 year and 10 month to 14 years,mean age of 10 years).Gastric tumor was found in 5 cases (interstitialoma in 1,lymphoma in 2,endodermal sinus tumor in 1 and P-J syndrome canceration in 1),whereas intestinal tumor was found in 24 cases(malignant Iymphoma in 17,colon carcinoma in 4 and carcinoid of appendix in 3).The main clinical manifestations were abdominal pain,emesia,fever,alimentary tract hemorrhage and emaciation.Surgical complication involved perforation,internal fistula,obstruction,invaginate and hemorrhage.Twenty-four patients underwent(gaster)intestine resection and anastomosis.Biopsy was performed in 5 patients.Two patients with colonic carcinoma relapsed 6 months to 1 year after operation.Of 29 patients,17 patients survived,6 patients missed the follow-up and 6 patients died.Conclusions Pediatric malignant gastrointestinal tumor is rare.The unspecific presentation makes the diagnosis difficult.In diagnosis of acute abdomen and gastrointestinal tract diseases,the possibility of malignant tumor should be reminded.The imaging and endoscopic examination are very important to diagnosis.The surgical resection or combined with chemotherapy are mainly used in treatment of tumors.The prognosis is related to the pathological nature of the tumor.

Effect of Fuzheng Jianpi on survival time in children solid tumors with chemotherapy / 肿瘤研究与临床

Objective To study the effect of traditional Chinese medicine on survival time in children solid tumors with chemotherapy. Methods 76 children with solid tumors, which were collected from January 2005 to March 2006, were randomly divided into treatment group (35 cases), control group (41 cases). The treatment group was treated with chemotherapy + self-dispensing Fuzheng Jianpi Chinese medicine. The control group was treated with chemotherapy alone. Results The survival rate of the treatment group was higher than that of the control group, the difference was statistically significant (P = 0.0024). The average survival time after chemotherapy in the treatment group was (31± 2) months (95 % CI 27-34 months), the control group was (21±2) months (95 % CI 16-25 months), which was shorter than the treatment group.Conclusion Chinese medicine and chemotherapy can prolong the survival time of children with solid tumors.

Diagnosis and treatment of solid-pseudopapillary tumor of the pancreas in children / 中华普通外科杂志

Objective To discuss the clinical manifestations, diagnosis and treatment of solidpseudopapillary tumor of the pancreas (SPTP) in children. Methods Clinical data of 12 cases of SPTP admitted to Beijing Children's Hospital from Mar 2001 to Mar 2007 were collected and analyzed retrospectively. Result There were 7 females and 5 males among the patients. The mean age was 13.6 years. The main symptoms were abdominal pain and mass. Tumors located in the head in 4 cases, in the body and tail of the pancreas in 8 cases. Five cases underwent tumor enucleations, one case underwent body resection of the pancreas, two cases underwent Whippple's procedure including one recurrent case, five cases underwent body and tail resection of the pancreas plus splenectomy. All these 12 cases were followed up for 6 months to 6 years. One case lost follow-up 6 months after body and tail resection of the pancreas plus spleneetomy. Another one suffered from live tumor one year after operation and refused surgical exploration and lost follow-up. The other 10 cases were still alive. Conclusion SPTP are predominantly seen in adolescent females. For its benign clinical behavior and relatively good prognosis, surgical resection is the main choice of therapy.

β-catenin mutation in pediatric malignant tumors / 国际外科学杂志

β-catenin is an important signaling transduetion and adhesion molecules.Mutation of the betacatenin gene,CTNNB1,is a common case in pediatric tumors,which may induce development and metastasis.In hepatoblastoma,48%have CTNNB1 mutation.In Wilms tumor,mutation only occurs in cases with WT1 gene mutation.In neuroblastoma,mutation is also found.