Gonçalves Fabrício Guimarães, Viaene Angela N, Vossough Arastoo
Division of Neuroradiology, Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, United States.
Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, PA, United States.
Front Neurol. 2021 Nov 10;12:733323. doi: 10.3389/fneur.2021.733323. eCollection 2021.
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.
即将出版的世界卫生组织中枢神经系统肿瘤分类第5版对弥漫性高级别胶质瘤(DHGGs)的术语进行了广泛修改。以前,“胶质母细胞瘤”作为一个描述性实体,可用于对小儿或成人DHGGs家族中的一些肿瘤进行分类。然而,现在“胶质母细胞瘤”这一术语已被剔除,不再用于小儿类型的DHGGs家族中的肿瘤。不过,作为一个实体,胶质母细胞瘤仍存在于成人弥漫性胶质瘤家族中,名为“IDH野生型胶质母细胞瘤”。值得注意的是,尽管胶质母细胞瘤在成人中更为常见,但在儿童中仍可检测到IDH野生型胶质母细胞瘤。尽管已从小儿类型的DHGGs家族中分离出来,但以前被标记为“小儿胶质母细胞瘤”的肿瘤仍以新的标签和新实体形式存在。由于分子生物学的进展,大多数以前所谓的“小儿胶质母细胞瘤”现在被归类为小儿类型的DHGGs四个家族成员之一。在本综述中,仍非正规地使用“胶质母细胞瘤”一词,主要是由于其历史相关性以及近期涉及新描述的新实体的文献较少。因此,这里使用“胶质母细胞瘤”作为一个统称,试图涵盖多个实体,如IDH突变型星形细胞瘤(4级)、IDH野生型胶质母细胞瘤、H3 G34突变型弥漫性半球胶质瘤、H3野生型和IDH野生型弥漫性小儿型高级别胶质瘤以及高级别婴儿型半球胶质瘤。胶质母细胞瘤是高度侵袭性肿瘤。它们可发生在发育中的中枢神经系统的任何部位,包括脊髓。体征和症状不具特异性,通常持续时间短,通常由颅内压升高或癫痫引起。也可能出现局部症状。“小儿胶质母细胞瘤”的治疗标准尚未明确确立,通常包括最大安全肿瘤切除手术。如果患者年龄大于3岁,建议随后进行放化疗。如果年龄小于3岁,则手术后继以化疗。一般来说,尽管进行了手术和辅助治疗,“小儿胶质母细胞瘤”的预后也很差。磁共振成像(MRI)是评估胶质母细胞瘤的首选成像方式。除了典型的传统MRI特征,即边界不清的高度异质性侵袭性肿块、对周围结构的占位效应以及不同程度的强化外,病变在实性成分中可能显示扩散受限、出血和灌注增加,反映血管生成增加。此外,磁共振波谱已被证明有助于术前和术后评估。最后,我们将提及已应用于评估成人胶质母细胞瘤且取得了有前景结果但在儿童中尚未广泛应用的新MRI技术。