Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Longitudinal Studies ; Male ; Neoplasm Staging ; Neuroblastoma ; diagnosis ; pathology ; therapy ; Treatment Outcome

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Neoplasms, Germ Cell and Embryonal ; diagnosis ; drug therapy ; pathology ; Ovarian Neoplasms ; diagnosis ; drug therapy ; Testicular Neoplasms ; diagnosis ; drug therapy

Objective To analyze the clinical characteristics and prognosis of 4 rare types of non -Hodgkin lymphoma(NHL)in children,and to discuss the progress in treatment.Methods Clinical data of 1 4 patients with rare types of NHL at Shanghai Children′s Medical Center,Shanghai Jiaotong University School of Medicine between January 2004 and December 201 4 were retrospectively analyzed,and their clinical features,treatment and prognosis were dis-cussed.Results Fourteen cases were reported including 6 subcutaneous panniculitis -like T -cell lymphoma (SPTCL),3 hydroa vacciniforme -like cutaneous lymphoma(HVLL),2 pediatric follicular lymphoma(PFL)and 3 ex-tranodal NK/T -cell lymphoma,and nasal type(ENKTL).Ten patients (71 .4%)primarily presented with skin lesions and underwent a long course of illness before they were finally diagnosed (the median was 1 0 months),71 .4%(1 0 /1 4 cases)of them associated with fever and 50.0%(7 /1 4 cases)with liver and spleen enlargement,and no evidence of central nervous system (CNS)and bone marrow (BM)involvement was observed,while 28.6% patients (4 /1 4 cases) had more than two lines′abnormalities in peripheral blood examination.Since there were no standard treatment guide-lines,most patients received CHOP (Cyclophosphamide +Adriamycin +Vincristine +Prednisone)and /or mature B -cell NHL -like therapy,and 50.0%(7 /1 4 cases)of them received interferon therapy in addition,while 1 patient re-ceived allogeneic hematopoietic stem cell transplantation after recurrence.The complete remission was achieved in 71 .4%(10 /14 cases)of all the patients.Two of them died,3 lost follow -up,and 1 relapsed.The 3 -year overall survi-val and event free survival rates were 0.84 and 0.57,respectively,after a median follow -up of 26 months (range 12 -64 months).Conclusions Pediatric rare types of NHL show atypical clinical manifestation,low incidence of CNS /BMinfil-tration and long course.It is hard to make pathological diagnosis and differentiation.It is also inappropriate to apply the commonly used staging system to these rare types of NHL.No standard treatment has been found by now.SPTCL,HVLL and PFL have relatively good outcomes when treated with mature B -cell NHL -type therapy plus interferon therapy.

Objective To investigate the organ function survival and late effects and secondary malignancy of childhood neuroblastoma (NB) with high intensity comprehensive treatment.Methods A total of 23 children with NB who received comprehensive treatment in Shanghai Children's Medical Center from December 1998 to October 2010 were enrolled.All tests were formulated according to the Children's Oncology Group (COG)'s guidelines with the approval of parents.The late side effects were graded by CTCAE v3.0,and hearing loss related to platinum was graded by Brock and Chang.Results The median follow-up was 96 (65-170) months.All the patients had at least one late side effect.The occurrence rate of 1-2 adverse effects was 100％,and 17.4％ (4/23 cases) of patients had grade 3-4 adverse effects.90.5％ of children (19/21 cases)developed hearing loss on both sides.Eleven children (52.4％,11/21 cases) developed dental abnormities,comprising microdontia,missing teeth,root stunting and enamel hypoplasia.9.5％ of children (2/12 cases) had scoliosis.42.8％ of children (9/12 cases) developed hypogonadism with the approval of parents or delayed growth.47.4％ of children (9/19 cases) had abnormal respiratory function.36.8％ of children (7/19 cases)had abnormal cardiac function.33.4％ of children (7/21 cases)developed renal damage.23.8 ％ of children (5/21 cases) had abnormal liver function.66.7％ of children (14/21 cases) had low level of cortisol,but adrenocorticotrophic hormone was normal.All children had normal function of thyroid.Three children developed secondary malignancy:leukemia,malignant fibroma and liver tumor.Conclusions The incidence of long-term adverse reactions of patients with neuroblastoma treated with high intensity comprehensive treatment is very high and the patients may have adverse effects,like hearing loss,dental abnormality,cardiopulmonary function abnormality,musculoskeletal problems and secondary malignancy.The upmost importance is to establish long-term follow-up program to detect the life quality of children and amend current therapeutic schemes improve life quality.

Objective To explore the management therapy of pediatric rhabdomyosarcoma.Methods Retrospective analysis of treatment of 29 cases with rhabdomyoscroma from Oct.1998 to Jun.2006.Primary sites included 5 cases of neck,1 chest wall,2 abdominal wall,12 retroperitoneal pelvic cavity and 9 extremity.Pathologic types included embryonal type 19(65.5%),alveolar 8(27.6%),pleomorphic 2(6.9%).According to IRS staging system they were Ⅰstage(n=2),Ⅱstage(n=6),Ⅲstage(n=9)and Ⅳstage(n=12).Most tumors were large than 5 cm(n=26).Results Twenty-seven cases were followed up but 2 of the cases were lost,19 cases got complete remission(4 cases relapsed or died after stopping treatment for 3 months to 3 years and 1 case relapsed after giving up treatment for 3 months),4 cases got partial remission(3 cases relapsed after 6 months diagnosed),4 cases got no response(3 of which deteriorated or relapsed and 1 case surved with tumor).Fourteen cases had constant remission between 5-77 months,averaging 22.9 months with average guitting time of treatment 16.9 months.Conclusion Total tumor resection,chemotherapy and radiotherapy play an important role in management of pediatric rhabdomyosarcoma.

Objective To study the diagnosis and treatment of the model of multidisciplinary team treatment on hepatoblastoma in children.Methods Retrospective analysis of treatment and long term follow-up of 16 patients with hepatoblastoma(8 cases were boys,8 cases were girls;aged 3 months to 11 years old,average age was 2 years old) during Aug.1998 to Jan.2006 in Shanghai Children's Medical Center.Special in multidisciplinary team treatment,preoperative evaluate and operation.Results The morbility locum of 8 cases were in right liver lobe,8 cases were in left liver lobe.Fifteen cases of the clinical presentation were abdominal mass,1 case was peritonitis.According to International Society of Pediatric Oncology(SIOP) Pretext staging system,43.7%(7/16)cases were stage Ⅱ,43.7%(7/16)cases were stage Ⅲ,12.5%(2/16)cases were stage Ⅳ.In 7 cases of stage Ⅱ,6 cases were accepted tumor total resection,1 case was accepted emergency surgery because tumor was fracture.Patients were given ICE protocal.In 7 cases of stage Ⅲ,6 cases were able to undergo delayed tumor resection following open biopsy and subsequent treatment with chemotherapy 2-3 courses.One case gave up treatment because its had poor response to chemotherapy.In 2 cases of stage Ⅳ,1 case was received liver transplantation due to poor respond to chemotherapy.One case was not received any treatment.In 7 cases of stage Ⅱ,6 cases were alive,1 case died,3-year free survival rate was 100%(4/4).In 7 cases of stage Ⅲ,5 cased were alive,3-year free survival rate was 75%(3/4).Two cases of stage Ⅳ all died.Conclusions Surgery play a very important role in the treatment of hepatoblastoma in children.Chemotherapy has improved the resectability of the tumor.Multidisciplinary team treatment is an effective model for diagnosis and treatment on childhood hepatoblastoma.

Objective To observe the relationship of IL-10 gene single nucleotide polymorphism and the susceptibility to ALL. Methods The bone marrow and peripheral blood samples from 115 ALL patients and 323 healthy controls were collected in Peking University First Hospital and Beijing Dao-pei Hospital from January 2007 to December 2009. The DNA were extracted from all samples. The primers of -819C/T and -592A/C in the promoter region of IL-10 gene were designed for the PCR. The restrictive fragment length polymorphism of IL-10 gene was analyzed by using restrictive enzyme Msl Ⅰ and HpyCH4 Ⅲ.Sequencing was done in part of these samples to confirm the results of PCR. The differences of genotypes and allele ratio of -819 and -592 sites were analyzed between the ALL patients and healthy controls. Real-time quantitative PCR was performed to detect the EB virus (EBV) infection and the expression of BCR/ABL fusion gene. The differences of genotypes and allele ratio of -819 and -592 sites were analyzed between the positive and negative group. Results The genotype ratios of -819CC, -819TT, - 819CT, -592AA,- 592CC and - 592AC were 14. 8% ( 17/115 ), 45.2% ( 52/115 ), 40. 0% ( 46/115 ), 43.5% ( 50/115 ),16. 5% ( 19/115 ), 40. 0% ( 46/115 ) in ALL patients, and were 9. 9% ( 32/323 ), 16. 4% ( 53/323 ),73.7% ( 238/323 ), 11.8% ( 38/323 ), 15.5% ( 50/323 ), 72. 8% ( 235/323 ) in the healthy controls,respectively. The genotypes of -819 and -592 sites had statistically significant differences between the two groups(x2 values were 46.000 and 54.550, all P ＜ 0. 05 ). The allele ratio of -819T and -592A were (65.2%, 150/230) and (63.5%, 146/230) in ALL patients, while they were 53.5% (344/646) and 48. 1% (311/646)in the healthy controls. There were statistically significant differences between the two groups (x2 values were 9. 877 and 15.986, all P ＜ 0. 05 ). The EBV DNA were detected in 42 ALL patients,among which 22 were positive and 20 were negative. The genotype ratios of -819CC, -819TT, -819CT,-592AA, - 592CC, - 592AC in EBV positive group were 9. 1% ( 2/22 ), 40. 9% ( 9/22 ), 50. 0%(11/22) ,31.8% ( 7/22 ), 13.6% ( 3/22 ), 54. 5% ( 12/22 ), while they were 35.0% ( 7/20 ), 45.0%(9/20) ,20. 0% (4/20) ,35.0% (7/20) ,45.0% (9/20) ,20. 0% (4/20) in the EBV negative group. The genotypes of -819 and -592 sites showed no statistical differences between the two groups( all P ＞ 0. 05 ).The BCR/ABL fusion gene were detected in 36 ALL patients, among which 20 were positive and 16 were negative. The genotype ratios of - 819CC, - 819TT, - 819CT, - 592AA, - 592CC, - 592AC in BCR/ABL positive group were 0% (0/20) ,45.0% (9/20) ,55.0% ( 11/20), 45. 0% (9/20) ,5.0% (1/20) ,50. 0%( 10/20), while they were 18. 8% ( 3/16 ), 50. 0% ( 8/16), 31.3% ( 5/16 ), 50. 0% ( 8/16 ), 18. 8%(3/16), 31.3 % (5/16)in the BCR/ABL negative group. The genotypes of -819 and -592 sites showed no statistical differences between the two groups ( all P ＞ 0. 05 ). Conclusion The population with - 819TT and - 592AA genotype of IL-10 gene shows susceptibility to ALL.

EBV (Epstein-Barr Virus) is a common herpes virus in patient with lymphatic system tumor, which firstly discovered in the cell line of Burkitt's lymphoma. 90% people worldwide had been infected by EB virus before grown-up, but not all people have the EBV-related disease or tumor. Most EBV infection can not elicit clinical symptoms. EBV infection in tumor of lymphatic system is common. It was early known that the EBV existence may cause the immortalization of normal B cells, which like the tumor cells. It means that EBV plays an important role in the tumorigenesis. EBV Bcrf1 code frame is homology to human IL-10, which is also called viral IL-10, showing immunosuppressive effect similar to the IL-10. IL-10 is also an important immunoregulatory factor, the secretory level of which influences the occurrence and development of lymphatic system diseases; the genotype of SNP site in IL-10 promoter region also associates with secretory level of IL-10. This review discusses the close relation between tumor of lymphatic system, EBV infection and gene polymorphism of IL-10.
Burkitt Lymphoma ; genetics ; virology ; Herpesvirus 4, Human ; genetics ; pathogenicity ; Humans ; Interleukin-10 ; genetics ; Lymphoma ; genetics ; virology ; Polymorphism, Single Nucleotide ; Viral Matrix Proteins ; genetics

Objectives To analyze the clinical features and long-term follow-up results of neuroblastoma (NB) without chemotherapy and radiotherapy, and to provide evidence for further improvement of treatment. Methods The clinical data of children diagnosed with NB who received operation during January 2005 to December 2015 was retrospectively analyzed, and the long-term follow-up results were evaluated. Results In 57 cases of NB, 43 cases (81.1％) were in stage 1, 8 cases were in stage 2 and 2 cases were in stage 4S. The median age at diagnose was 7 months (11 days - 10 years and 11 months). There were 47/51 cases had the pathological type with a good prognosis (accounting for 92.2％). FISH was detected in 1/49 case which had the amplification at greater than 10 copies. 56/57 cases underwent surgical resection of the primary tumor, 50 cases of which were completely resected, and 3 cases had very good partial remission after tumor resection. The abdominal mass was found in the uterus in 1 case, and surgical operation was not performed and the imaging was regularly checked for follow-up , and the mass subsided completely at 7-month-old. The median follow-up time was 36 months (4 - 99 months). Five children were lost to follow-up and the median time of follow-up was 19 months (4 - 45 months). One child in stage 4S relapsed at 1 year of follow-up, 2 cases in stage 1 relapsed at 6 months of follow-up. Five years event free survival rates (EFS) in all patients were 94.6％, and overall survival rate (OS) of the 5 years was 100％. Conclusions Children younger than 18 months without MYCN amplification in the stages 1 and 2 are safe by surgical treatment alone with good prognosis. Simple surgical treatment can also be extended to all age groups of NB without MYCN amplification in the stages 1 or 2.

Objective To evaluate the outcomes of children with stage Ⅳ malignant extracranial germ cell tumors. Methods Twenty-five patients were enrolled in the retrospective analysis. Event-free survival (EFS) and overall survival (OS) rates were estimated by Kaplan-Meier method with SPSS 13.0. Results Of the 25 children, there were 13 males and 12 females. The mean age at diagnosis was 2 years old (ranged 1 to 11). Five patients receiving chemotherapy in another hospital before (n=1), or giving up treatment after confirmed diagnosis (n=1), or giving up effective treatment after received less than 2 cycles (n=3) were excluded from this analysis. Of the 20 patients, 90.0% (18/20) achieved complete remission and 5.0% (1/20) achieved partial remission after treatment. The 5-year EFS rate and 5-year OS rate were 70.0%±10.2% and 82.4%±9.2% respectively. There was no death occurred due to complications. Conclusions The effect of this treatment program is positive. The cumulative dose of the drugs is not high, compared with other schemes such as PEB, but there are more drugs involved. Whether these drugs may cause long-term adverse reactions needs further research.