Urbańska Kaja, Sokołowska Justyna, Szmidt Maciej, Sysa Paweł
Division of Histology and Embryology, Department of Morphological Science, Faculty of Veterinary Medicine, Warsaw University of Life Sciences-SGGW, Warsaw, Poland.
Contemp Oncol (Pozn). 2014;18(5):307-12. doi: 10.5114/wo.2014.40559. Epub 2014 Aug 4.
Glioblastoma multiforme is a central nervous system tumor of grade IV histological malignancy according to the WHO classification. Over 90% of diagnosed glioblastomas multiforme cases are primary gliomas, arising from normal glial cells through multistep oncogenesis. The remaining 10% are secondary gliomas originating from tumors of lower grade. These tumors expand distinctly more slowly. Although genetic alterations and deregulations of molecular pathways leading to both primary and secondary glioblastomas formation differ, morphologically they do not reveal any significant differences. Glioblastoma is a neoplasm that occurs spontaneously, although familial gliomas have also been noted. Caucasians, especially those living in industrial areas, have a higher incidence of glioblastoma. Cases of glioblastoma in infants and children are also reported. The participation of sex hormones and viruses in its oncogenesis was also suggested. Progression of glioblastoma multiforme is associated with deregulation of checkpoint G1/S of a cell cycle and occurrence of multiple genetic abnormalities of tumor cells. Metastases of glioblastoma multiforme are rarely described. Treatment of glioblastoma multiforme includes tumor resection, as well as radiotherapy and chemotherapy. Drugs inhibiting integrin signaling pathways and immunotherapy are also employed. Treatment modalities and prognosis depend on the tumor localization, degree of its malignancy, genetic profile, proliferation activity, patient's age and the Karnofsky performance scale score. Although the biology of glioblastoma multiforme has recently been widely investigated, the studies summarizing the knowledge of its development and treatment are still not sufficient in terms of comprehensive brain tumor analysis.
根据世界卫生组织的分类,多形性胶质母细胞瘤是一种组织学恶性程度为IV级的中枢神经系统肿瘤。超过90%被诊断为多形性胶质母细胞瘤的病例是原发性胶质瘤,通过多步骤肿瘤发生过程由正常神经胶质细胞产生。其余10%是源自低级别肿瘤的继发性胶质瘤。这些肿瘤的生长明显更缓慢。尽管导致原发性和继发性胶质母细胞瘤形成的基因改变和分子途径失调有所不同,但在形态学上它们没有显示出任何显著差异。胶质母细胞瘤是一种自发发生的肿瘤,尽管也有家族性胶质瘤的报道。高加索人,尤其是生活在工业区的人,胶质母细胞瘤的发病率较高。也有婴儿和儿童患胶质母细胞瘤的病例报道。还提出了性激素和病毒在其肿瘤发生中的作用。多形性胶质母细胞瘤的进展与细胞周期G1/S检查点的失调以及肿瘤细胞的多种基因异常的发生有关。多形性胶质母细胞瘤的转移很少被描述。多形性胶质母细胞瘤的治疗包括肿瘤切除以及放疗和化疗。也使用抑制整合素信号通路的药物和免疫疗法。治疗方式和预后取决于肿瘤的定位、恶性程度、基因谱、增殖活性、患者年龄和卡诺夫斯基功能状态评分。尽管最近对多形性胶质母细胞瘤的生物学进行了广泛研究,但就全面的脑肿瘤分析而言,总结其发展和治疗知识的研究仍然不足。