Madden Jennifer R, Addo-Yobo Steven O, Donson Andrew M, Liu Arthur K, McNatt Sean A, Kleinschmidt-Demasters B K, Fenton Laura Z, Foreman Nicholas K, Smith Amy A
Department of Neuro-Oncology, The Children's Hospital, Aurora, CO 80045, USA.
J Pediatr Hematol Oncol. 2010 Oct;32(7):e272-8. doi: 10.1097/MPH.0b013e3181e51403.
Outcomes for average-risk medulloblastoma are excellent with 5-year event-free survival and overall survival>80%. Treatment failures include radiation-induced glioblastomas (RIG), which are often diagnosed solely on imaging. Recent studies suggest that RIGs differ from spontaneous glioblastoma multiforme (GBM), based on microarray gene-expression profiling. Retrospective review of children with average-risk medulloblastoma treated from 1996 to 2003 included 16 patients with 5 treatment failures. One died of disease progression, 1 died as a result of radiation necrosis, and 3 children died of pathology-confirmed GBM. Of these 3 GBMs, one was studied with electron microscopy, cytogenetics, and gene-expression microarray analysis. This tumor had focal medulloblastoma and similarity by gene-expression microarray with other RIGs. With both components in the recurrent tumor, we suggest it was in the process of transitioning from medulloblastoma to RIG, that is, "catching the tumor in the act." Some radiation-induced nervous system tumors may develop as a direct result of severe oncologic changes within the original tumor cells, with the tumor evolving into a different phenotypic tumor type. We recommend biopsy for tissue confirmation and genetic expression profile to shed light on the etiology of radiation-induced neoplasms.
低风险髓母细胞瘤患者的预后良好,5年无事件生存率和总生存率均超过80%。治疗失败包括放射性诱导的胶质母细胞瘤(RIG),这类肿瘤通常仅通过影像学诊断。最近的研究表明,基于基因芯片基因表达谱分析,RIG与自发性多形性胶质母细胞瘤(GBM)不同。对1996年至2003年接受治疗的低风险髓母细胞瘤患儿进行回顾性研究,纳入16例出现5次治疗失败的患者。1例死于疾病进展,1例死于放射性坏死,3例儿童死于病理确诊的GBM。在这3例GBM中,对其中1例进行了电子显微镜检查、细胞遗传学分析和基因表达芯片分析。该肿瘤具有局灶性髓母细胞瘤成分,通过基因表达芯片分析显示与其他RIG相似。鉴于复发性肿瘤中存在这两种成分,我们认为它正处于从髓母细胞瘤向RIG转变的过程中,即“当场捕获肿瘤”。一些放射性诱导的神经系统肿瘤可能是原始肿瘤细胞内严重肿瘤学改变的直接结果,肿瘤演变成不同表型的肿瘤类型。我们建议进行活检以进行组织确认和基因表达谱分析,从而阐明放射性诱导肿瘤的病因。