Gliomas in neurofibromatosis type 1: A clinicopathologic study of 100 patients
Gliomas in neurofibromatosis type 1: A clinicopathologic study of 100 patients
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DOI：
10.1097/nen.0b013e318165eb75
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发表时间：
2008-03-01
影响因子：
3.2
通讯作者：
Giannini, Caterina
中科院分区：
文献类型：
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作者：
Rodriguez, Fausto J.;Perry, Arie;Giannini, Caterina
1型神经纤维瘤病患者胶质瘤的病理研究很少。我们分析了100例1型神经纤维瘤病患者的临床和病理特征（男性57例，女性43例）。肿瘤诊断时的中位年龄为13岁（范围4个月至68岁）。大多数肿瘤为典型的毛细胞性星形细胞瘤（PA）（49%）或弥漫性浸润性星形细胞瘤（DA）（27%），包括世界卫生组织分级II级（5%）、III级（15%）和IV级（7%）；其余为低级别星形细胞瘤，亚型不确定（LGSI; 17%）。2例毛粘液样星形细胞瘤，1例结缔组织增生婴儿神经节胶质瘤和1例常规神经节胶质瘤。24例肿瘤发生于视神经通路，包括PA（14例）、LGSI（4例）、DA（4例）、毛粘液样星形细胞瘤（1例）、神经节胶质瘤（1例）。PA和LGSI胶质瘤的预后总体良好；基于部位、肿瘤大小、有丝分裂活性或MIB-1标记指数，PA组和LGSI组之间没有生存差异。在PA和LGSI联合组中，年龄小于10岁和总切除与总生存率增加相关（p分别= 0.047和0.002）。与联合组（PA + LGSI）相比，所有部位DA患者的总生存时间和无复发生存时间均缩短（p < 0.001和p = 0.003）。本研究强调了1型神经纤维瘤患者中发生的神经胶质瘤的广泛组织学谱。经典PA和LGSI是最常见的，大多数预后良好。相比之下，da更具攻击性，类似于那些零星出现的da。
There are few pathologic studies of gliomas in patients with neurofibromatosis type 1. We analyzed clinical and pathologic features of gliomas from 100 neurofibromatosis type 1 patients (57 men; 43 women). The median age at tumor diagnosis was 13 years (range, 4 months to 68 years). Most tumors were typical pilocytic astrocytoma (PA) (49%) or diffusely infiltrating astrocytoma (DA) (27%) that included World Health Organization Grades II (5%), III (15%), and IV (7%); others were designated as low-grade astrocytoma, subtype indeterminate (LGSI; 17%). Two pilomyxoid astrocytomas, 1 desmoplastic infantile ganglioglioma and 1 conventional ganglioglioma, were also identified. The tumors in 24 cases arose in the optic pathways and included PA (n = 14), LGSI (n = 4), DA (n = 4), pilomyxoid astrocytoma (n = 1), and ganglioglioma (n = 1). The prognoses of the PA and LGSI gliomas overall were generally favorable; there were no survival differences between PA and LGSI groups based on site, tumor size, mitotic activity, or MIB-1 labeling index. In the combined PA and LGSI group, age younger than 10 years and gross total resection were associated with an increased overall survival rate (p = 0.047 and 0.002, respectively). Compared with the combined group (PA + LGSI), patients with DA at all sites had decreased overall and recurrence-free survival times (p < 0.001 and p = 0.003, respectively). This study emphasizes the wide histologic spectrum of gliomas that occur in patients with neurofibromatosis type 1. Classic PA and LGSI are the most common, and most have favorable prognoses. By contrast, DAs are more aggressive, similar to those that arise sporadically.