即将发布的第5版世界卫生组织中枢神经系统肿瘤分类正在带来弥漫性高级别神经胶质瘤(DHGG)术语的广泛变化。以前的胶质母细胞瘤,“作为描述性实体,可以用于对儿科或成人DHGs家族的一些肿瘤进行分类。然而,现在,术语“胶质母细胞瘤”已被删除,不再适用于儿科DHGs家族中的肿瘤。作为一个实体,胶质母细胞瘤仍然存在,然而,在“胶质母细胞瘤”标志下的成人类型的弥漫性神经胶质瘤家族中,IDH-野生型。\"值得注意的是,胶质母细胞瘤仍然可以在儿童中检测到,在这个种群中发现了IDH-野生型,尽管在成年人中更为常见。尽管与儿科类型的DHGs家族分离,以前被标记为“小儿胶质母细胞瘤”的东西仍然具有新的标签和新的实体。由于分子生物学的进步,以前被称为“小儿胶质母细胞瘤”的大多数现在被归类为小儿DHGs的四个家族成员之一。在这次审查中,胶质母细胞瘤一词仍被伪造使用,主要是由于其历史相关性以及最近缺乏涉及最近描述的新实体的最新文献。因此,“胶质母细胞瘤”在这里被用作一个总称,试图涵盖多个实体,如星形细胞瘤,IDH突变体(4级);胶质母细胞瘤,IDH-野生型;弥漫性半球神经胶质瘤,H3G34突变型;弥漫性小儿型高级别胶质瘤,H3-野生型和IDH-野生型;和高级别婴儿型半球神经胶质瘤。胶质母细胞瘤是高度侵袭性肿瘤。它们可能出现在发育中的中枢神经系统的任何地方,包括脊髓.体征和症状是非特异性的,通常持续时间短,通常源于颅内压升高或癫痫发作。也可能出现局部症状。“小儿胶质母细胞瘤”的护理标准并不完善,通常由具有最大安全肿瘤切除的手术组成。如果患者年龄超过3岁,则建议进行随后的放化疗。如果小于3岁,手术后是化疗。总的来说,尽管手术和辅助治疗,“小儿胶质母细胞瘤”的预后也较差。磁共振成像(MRI)是评估胶质母细胞瘤的首选成像方式。除了典型的常规MRI特征外,即,边界模糊的高度异质侵入性肿块,对周围结构的质量影响,和可变程度的增强,病变可能显示固体成分的扩散受限,出血,和增加灌注,反映增加的血管和血管生成。此外,磁共振波谱已被证明有助于术前和术后评估。最后,我们将参考新的MRI技术,已经被用于评估成人胶质母细胞瘤,有希望的结果,还没有在儿童中广泛使用。
The shortly upcoming 5th edition of the World Health Organization Classification of Tumors of the Central Nervous System is bringing extensive changes in the terminology of diffuse high-grade gliomas (DHGGs). Previously \"glioblastoma,\" as a descriptive entity, could have been applied to classify some tumors from the family of pediatric or adult DHGGs. However, now the term \"glioblastoma\" has been divested and is no longer applied to tumors in the family of pediatric types of DHGGs. As an entity, glioblastoma remains, however, in the family of adult types of diffuse gliomas under the insignia of \"glioblastoma, IDH-wildtype.\" Of note, glioblastomas still can be detected in children when glioblastoma, IDH-wildtype is found in this population, despite being much more common in adults. Despite the separation from the family of pediatric types of DHGGs, what was previously labeled as \"pediatric glioblastomas\" still remains with novel labels and as new entities. As a result of advances in molecular biology, most of the previously called \"pediatric glioblastomas\" are now classified in one of the four family members of pediatric types of DHGGs. In this review, the term glioblastoma is still apocryphally employed mainly due to its historical relevance and the paucity of recent literature dealing with the recently described new entities. Therefore, \"glioblastoma\" is used here as an umbrella term in the attempt to encompass multiple entities such as astrocytoma, IDH-mutant (grade 4); glioblastoma, IDH-wildtype; diffuse hemispheric glioma, H3 G34-mutant; diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype; and high grade infant-type hemispheric glioma. Glioblastomas are highly aggressive neoplasms. They may arise anywhere in the developing central nervous system, including the spinal cord. Signs and symptoms are non-specific, typically of short duration, and usually derived from increased intracranial pressure or seizure. Localized symptoms may also occur. The standard of care of \"pediatric glioblastomas\" is not well-established, typically composed of surgery with maximal safe tumor resection. Subsequent chemoradiation is recommended if the patient is older than 3 years. If younger than 3 years, surgery is followed by chemotherapy. In general, \"pediatric glioblastomas\" also have a poor prognosis despite surgery and adjuvant therapy. Magnetic resonance imaging (MRI) is the imaging modality of choice for the evaluation of glioblastomas. In addition to the typical conventional MRI features, i.e., highly heterogeneous invasive masses with indistinct borders, mass effect on surrounding structures, and a variable degree of enhancement, the lesions may show restricted diffusion in the solid components, hemorrhage, and increased perfusion, reflecting increased vascularity and angiogenesis. In addition, magnetic resonance spectroscopy has proven helpful in pre- and postsurgical evaluation. Lastly, we will refer to new MRI techniques, which have already been applied in evaluating adult glioblastomas, with promising results, yet not widely utilized in children.