El-Ayadi Moatasem, Ansari Marc, Sturm Dominik, Gielen Gerrit H, Warmuth-Metz Monika, Kramm Christof M, von Bueren Andre O
Department of Pediatrics and Adolescent Medicine, Division of Pediatric Hematology and Oncology, University Hospital of Geneva, Geneva, Switzerland.
Department of Pediatrics, CANSEARCH Research Laboratory, Faculty of Medicine, University of Geneva, Geneva, Switzerland.
Oncotarget. 2017 Jun 14;8(38):64564-64578. doi: 10.18632/oncotarget.18478. eCollection 2017 Sep 8.
In the past years, pediatric high-grade gliomas (HGG) have been the focus of several research articles and reviews, given the recent discoveries on the genetic and molecular levels pointing out a clinico-biological uniqueness of the pediatric population compared to their adult counterparts with HGG. On the other hand, there are only scarce data about HGG in very young children (below 3 years of age at diagnosis) due to their relatively low incidence. However, the few available data suggest further distinction of this very rare subgroup from older children and adults at several levels including their molecular and biological characteristics, their treatment management, as well as their outcome. This review summarizes and discusses the current available knowledge on the epidemiological, neuropathological, genetic and molecular data of this subpopulation. We discuss these findings and differences compared to older patients suffering from the same histologic disease. In addition, we highlight the particular clinical and neuro-radiological findings in this specific subgroup of patients as well as their current management approaches and treatment outcomes.
在过去几年中，儿童高级别胶质瘤（HGG）一直是多篇研究文章和综述的焦点，因为最近在基因和分子水平上的发现指出，与患有HGG的成人相比，儿童群体具有临床生物学独特性。另一方面，关于极年幼患儿（诊断时年龄小于3岁）的HGG数据非常稀少，因为其发病率相对较低。然而，现有的少量数据表明，这个非常罕见的亚组在包括分子和生物学特征、治疗管理以及预后等多个层面上与年龄较大的儿童和成人存在进一步差异。本综述总结并讨论了关于这一亚群体的流行病学、神经病理学、基因和分子数据的现有知识。我们将这些发现以及与患有相同组织学疾病的年长患者相比的差异进行了讨论。此外，我们强调了这一特定亚组患者的特殊临床和神经放射学发现，以及他们目前的管理方法和治疗结果。
