Theeler Brett J, Ellezam Benjamin, Melguizo-Gavilanes Isaac, de Groot John F, Mahajan Anita, Aldape Kenneth D, Bruner Janet M, Puduvalli Vinay K
Department of Neurology and John P. Murtha Cancer Center, Walter Reed National Military Medical Center, 8901 Wisconsin Avenue, Building 19, Bethesda, MD 20889, United States.
Department of Pathology, CHU Sainte-Justine, Universite de Montreal, 3175 Cote-Ste-Catherine Rd, Montreal, Quebec H3TIC5, Canada.
J Neurol Sci. 2015;353(1-2):92-7. doi: 10.1016/j.jns.2015.04.014. Epub 2015 Apr 18.
Brainstem gliomas are rare in adults and overall have superior survival outcomes compared to pediatric brainstem gliomas.
We conducted a retrospective data and tissue analysis of all adult patients (≥ 18 years old) with World Health Organization (WHO) Grade II, III, and IV brainstem gliomas in the University of Texas MD Anderson Cancer Center institutional database from 1990 to 2012.
We identified 143 cases in adults ages 18 and over. There were 28 glioblastomas, 43 anaplastic astrocytomas, 15 diffuse astrocytomas, and 11 gliomas not otherwise specified, and in 46 cases the diagnosis was made radiographically. 128 (89.5%) cases were classified radiographically as diffuse and of the focal tumors, 9 of the 15 were WHO Grade III or IV tumors. Increasing tumor grade and contrast enhancement were associated with significantly reduced overall survival. The median overall survival for the entire cohort was 32.1 months similar to previously published studies. Two of 25 grade II and III tumors, and 1 of 17 glioblastomas had IDH1 mutations on immunohistochemical testing. Nine cases had sufficient tissue for mutation profiling, 1 case had a BRAF V600E mutation and 2 had 2 PIK3CA mutations.
Survival outcomes for adult WHO Grade II to IV brainstem gliomas were similar to supratentorial IDH1 wild-type tumors of similar grade and histology. Potentially actionable mutations can be identified from small biopsy samples in a subset of adult brainstem gliomas.
脑干胶质瘤在成人中较为罕见,总体生存结果优于儿童脑干胶质瘤。
我们对1990年至2012年德克萨斯大学MD安德森癌症中心机构数据库中所有年龄≥18岁的世界卫生组织(WHO)二级、三级和四级脑干胶质瘤成年患者进行了回顾性数据和组织分析。
我们确定了18岁及以上成人的143例病例。其中有28例胶质母细胞瘤、43例间变性星形细胞瘤、15例弥漫性星形细胞瘤和11例未另行指定的胶质瘤,46例通过影像学诊断。128例(89.5%)在影像学上被分类为弥漫性,在局灶性肿瘤中,15例中有9例为WHO三级或四级肿瘤。肿瘤分级增加和增强扫描与总体生存率显著降低相关。整个队列的中位总生存期为32.1个月,与先前发表的研究相似。25例二级和三级肿瘤中有2例,17例胶质母细胞瘤中有1例在免疫组化检测中存在异柠檬酸脱氢酶1(IDH1)突变。9例有足够的组织进行突变分析,1例有BRAF V600E突变,2例有2个磷脂酰肌醇-3激酶催化亚基α(PIK3CA)突变。
成人WHO二级至四级脑干胶质瘤的生存结果与幕上类似分级和组织学的IDH1野生型肿瘤相似。在一部分成人脑干胶质瘤中,可以从小活检样本中识别出潜在的可操作突变。