Clinical characteristics and prognosis of pediatric medulloblastoma: a case study of 40 patients at children’s hospital of Soochow university

Pediatric medicine (Hong Kong, China) Pub Date : 2021-01-01 DOI:10.21037/pm-21-59

Weiwei Du, Ye Lu, Fan Yang, Q. Lu, Zhiheng Li, Jun Lu, Shaoyan Hu

{"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}

引用次数: 0

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

查看原文本刊更多论文

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

背景：探讨儿童髓母细胞瘤（MB）的临床、组织学和分子特征及其预后。方法：作者分析了2011年11月至2020年10月期间东吴大学儿童医院手术切除后经病理诊断为MB患者的临床和病理数据。结果：共考虑了40名患者（20名男性和20名女性），其中MB发病的中位年龄为81个月（5-163个月）。其中，完全切除26例（26/40，65%），次全切除14例（14/40，35%）。切除后病理学检查显示8例MB已经转移。所有40名儿童都进行了组织学分类，确定：经典型32例（32/40，80%），结缔组织增生性/结节型6例（6/40，15%），间变性型2例（2/40，5%）。对19名儿童进行了分子分型测试，确定：无翼激活（WNT激活）组1例（1/19，5.3%），声波刺猬激活（SHH激活）组6例（6/19，31.6%），第3组7例（7/19，36.8%），第4组5例（5/19，26.3%）。在40名患者中，29人接受了手术、放疗和化疗的联合治疗，中位随访时间为25个月（1-102个月）。3年总生存率（OS）和无事件生存率（EFS）分别为（64.3±10.4）%和（61.8±10.3）%。单因素分析显示年龄、临床分期、病理亚型、，Cox回归分析显示年龄和肿瘤转移是儿童MB预后不良的独立危险因素（P<0.05）。结论：转移和初诊年龄是影响儿童MB预后的不利因素；放疗可改善MB患者的预后。8.

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

Pediatric medicine (Hong Kong, China)

CiteScore

1.20

自引率

0.00%

发文量