Brandes Alba A, Paris Myriam K, Basso Umberto
Department of Medical Oncology, University Hospital, Padova, Italy.
Expert Rev Anticancer Ther. 2003 Oct;3(5):615-20. doi: 10.1586/14737140.3.5.615.
The origin and histological classification of embryonal tumors, which make up the largest group of malignant pediatric brain malignancies, are two controversial issues. However, in recent years, progress has been made in our understanding of the molecular genetic abnormalities that govern their onset and/or progression. Several of these abnormalities appear to involve alterations in the signaling system controlling normal cerebellar development. An improved understanding of both the biology and clinical relevance of these molecular defects is probably the only means by which neuro-oncologists will improve the management of these tumors, thus reducing disease- and treatment-related morbidity and mortality.
构成小儿恶性脑肿瘤最大群体的胚胎性肿瘤的起源和组织学分类是两个有争议的问题。然而,近年来,我们对控制其发生和/或进展的分子遗传异常的理解取得了进展。其中一些异常似乎涉及控制正常小脑发育的信号系统的改变。更好地理解这些分子缺陷的生物学特性和临床相关性可能是神经肿瘤学家改善这些肿瘤治疗的唯一途径,从而降低疾病和治疗相关的发病率和死亡率。