Biegel J A
Division of Human Genetics and Molecular Biology, Children's Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.
Neuro Oncol. 1999 Apr;1(2):139-51. doi: 10.1093/neuonc/1.2.139.
Considerable progress has been made toward improving survival for children with brain tumors, and yet there is still relatively little known regarding the molecular genetic events that contribute to tumor initiation or progression. Nonrandom patterns of chromosomal deletions in several types of childhood brain tumors suggest that the loss or inactivation of tumor suppressor genes are critical events in tumorigenesis. Deletions of chromosomal regions 10q, 11 and 17p, and example, are frequent events in medulloblastoma, whereas loss of a region within 22q11.2, which contains the INI1 gene, is involved in the development of atypical teratoid and rhabdoid tumors. A review of the cytogenetic and molecular genetic changes identified to date in childhood brain tumors will be presented.
在提高脑肿瘤患儿的生存率方面已取得了显著进展，然而，对于导致肿瘤发生或进展的分子遗传事件，我们仍然知之甚少。几种儿童脑肿瘤中染色体缺失的非随机模式表明，肿瘤抑制基因的缺失或失活是肿瘤发生中的关键事件。例如，染色体区域10q、11和17p的缺失在髓母细胞瘤中是常见事件，而22q11.2区域内包含INI1基因的缺失则与非典型畸胎样和横纹肌样肿瘤的发生有关。本文将对迄今为止在儿童脑肿瘤中发现的细胞遗传学和分子遗传学变化进行综述。
