Weiwei Du, Ye Lu, Fan Yang, Q. Lu, Zhiheng Li, Jun Lu, Shaoyan Hu
{"title":"苏州大学儿童医院40例小儿髓母细胞瘤临床特点及预后分析","authors":"Weiwei Du, Ye Lu, Fan Yang, Q. Lu, Zhiheng Li, Jun Lu, Shaoyan Hu","doi":"10.21037/pm-21-59","DOIUrl":null,"url":null,"abstract":"Background: To explore the clinical, histological, and molecular characteristics of childhood medulloblastoma (MB) and its prognosis. Methods: The authors analyzed clinical and pathological data from MB patients at Children’s Hospital of Soochow University who were diagnosed by pathology after surgical resection between November 2011 and October 2020. Results: A total of 40 patients were considered (20 males and 20 females), where the median age at MB onset was 81 months (5–163 months). Of these, complete resection was performed for 26 cases (26/40, 65%) and subtotal resection performed for 14 cases (14/40, 35%). Pathology after resection indicated that in 8 cases the MB had already metastasized. All 40 children were histologically classified, identifying: 32 cases of classic type (32/40, 80%), 6 cases of desmoplastic/nodular type (6/40, 15%), and 2 cases of anaplastic type (2/40, 5%). Molecular typing tests were performed on 19 children, identifying: 1 case in the wingless-activated (WNT-activated) group (1/19, 5.3%), 6 cases in the sonic hedgehog-activated (SHH-activated) group (6/19, 31.6%), 7 cases in Group 3 (7/19, 36.8%), and 5 cases in Group 4 (5/19, 26.3%). Of the 40 patients, 29 received a combined treatment of surgery, radiotherapy, and chemotherapy, and the median follow-up time was 25 months (1–102 months). The 3-year overall survival rate (OS) and event-free survival rate (EFS) were (64.3±10.4)% and (61.8±10.3)%, respectively. Univariate analyses showed that age, clinical stage, pathological subtype, and radiotherapy were potentially relevant variables in patient prognosis (P<0.05). Cox regression analysis showed that age and tumor metastasis were independent risk factors for poor prognosis of children with MB (P<0.05). Conclusions: Metastasis and age at initial diagnosis are unfavorable factors in the prognosis of childhood MB patients; however, radiotherapy could improve the prognosis of MB patients. 8","PeriodicalId":74411,"journal":{"name":"Pediatric medicine (Hong Kong, China)","volume":" ","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Clinical characteristics and prognosis of pediatric medulloblastoma: a case study of 40 patients at children’s hospital of Soochow university\",\"authors\":\"Weiwei Du, Ye Lu, Fan Yang, Q. Lu, Zhiheng Li, Jun Lu, Shaoyan Hu\",\"doi\":\"10.21037/pm-21-59\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Background: To explore the clinical, histological, and molecular characteristics of childhood medulloblastoma (MB) and its prognosis. Methods: The authors analyzed clinical and pathological data from MB patients at Children’s Hospital of Soochow University who were diagnosed by pathology after surgical resection between November 2011 and October 2020. Results: A total of 40 patients were considered (20 males and 20 females), where the median age at MB onset was 81 months (5–163 months). Of these, complete resection was performed for 26 cases (26/40, 65%) and subtotal resection performed for 14 cases (14/40, 35%). Pathology after resection indicated that in 8 cases the MB had already metastasized. All 40 children were histologically classified, identifying: 32 cases of classic type (32/40, 80%), 6 cases of desmoplastic/nodular type (6/40, 15%), and 2 cases of anaplastic type (2/40, 5%). Molecular typing tests were performed on 19 children, identifying: 1 case in the wingless-activated (WNT-activated) group (1/19, 5.3%), 6 cases in the sonic hedgehog-activated (SHH-activated) group (6/19, 31.6%), 7 cases in Group 3 (7/19, 36.8%), and 5 cases in Group 4 (5/19, 26.3%). Of the 40 patients, 29 received a combined treatment of surgery, radiotherapy, and chemotherapy, and the median follow-up time was 25 months (1–102 months). The 3-year overall survival rate (OS) and event-free survival rate (EFS) were (64.3±10.4)% and (61.8±10.3)%, respectively. Univariate analyses showed that age, clinical stage, pathological subtype, and radiotherapy were potentially relevant variables in patient prognosis (P<0.05). Cox regression analysis showed that age and tumor metastasis were independent risk factors for poor prognosis of children with MB (P<0.05). Conclusions: Metastasis and age at initial diagnosis are unfavorable factors in the prognosis of childhood MB patients; however, radiotherapy could improve the prognosis of MB patients. 8\",\"PeriodicalId\":74411,\"journal\":{\"name\":\"Pediatric medicine (Hong Kong, China)\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2021-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Pediatric medicine (Hong Kong, China)\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.21037/pm-21-59\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatric medicine (Hong Kong, China)","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.21037/pm-21-59","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Clinical characteristics and prognosis of pediatric medulloblastoma: a case study of 40 patients at children’s hospital of Soochow university
Background: To explore the clinical, histological, and molecular characteristics of childhood medulloblastoma (MB) and its prognosis. Methods: The authors analyzed clinical and pathological data from MB patients at Children’s Hospital of Soochow University who were diagnosed by pathology after surgical resection between November 2011 and October 2020. Results: A total of 40 patients were considered (20 males and 20 females), where the median age at MB onset was 81 months (5–163 months). Of these, complete resection was performed for 26 cases (26/40, 65%) and subtotal resection performed for 14 cases (14/40, 35%). Pathology after resection indicated that in 8 cases the MB had already metastasized. All 40 children were histologically classified, identifying: 32 cases of classic type (32/40, 80%), 6 cases of desmoplastic/nodular type (6/40, 15%), and 2 cases of anaplastic type (2/40, 5%). Molecular typing tests were performed on 19 children, identifying: 1 case in the wingless-activated (WNT-activated) group (1/19, 5.3%), 6 cases in the sonic hedgehog-activated (SHH-activated) group (6/19, 31.6%), 7 cases in Group 3 (7/19, 36.8%), and 5 cases in Group 4 (5/19, 26.3%). Of the 40 patients, 29 received a combined treatment of surgery, radiotherapy, and chemotherapy, and the median follow-up time was 25 months (1–102 months). The 3-year overall survival rate (OS) and event-free survival rate (EFS) were (64.3±10.4)% and (61.8±10.3)%, respectively. Univariate analyses showed that age, clinical stage, pathological subtype, and radiotherapy were potentially relevant variables in patient prognosis (P<0.05). Cox regression analysis showed that age and tumor metastasis were independent risk factors for poor prognosis of children with MB (P<0.05). Conclusions: Metastasis and age at initial diagnosis are unfavorable factors in the prognosis of childhood MB patients; however, radiotherapy could improve the prognosis of MB patients. 8