Department of Neurosurgery, Keio University School of Medicine, Tokyo, Japan.
J Neurooncol. 2012 May;108(1):37-44. doi: 10.1007/s11060-012-0797-4. Epub 2012 Jan 25.
With the exception of radiation-induced tumors, benign meningiomas that are known to have developed within a defined time period are extremely rare. We have genetically characterized two cases of radiographically defined de novo, sporadic meningiomas--a 5-cm, left parasagittal tumor in a 61-year-old male and a 2.3-cm, right falx tumor in a 53-year-old female. Neither tumor was observed during MRIs performed for unrelated complaints 49 and 28 months before surgery, respectively. Both tumors were totally resected, and histopathological examination revealed WHO grade I meningiomas. In both cases, the MIB-1 staining indices were high for grade I meningioma (5.6% for case 1 and 9.1% for case 2), and abnormal accumulation of p53 were observed by immunohistochemistry. The two tumors shared losses of chromosome arms 1p and 7p by comparative genomic hybridization. The tumor suppressor merlin, product of the NF2 gene, was not detected in either tumor. These abnormalities found in common in both of the de novo meningiomas likely to play significant roles in the pathogenesis and/or rapid development of meningiomas. Moreover, taken together with previous studies, our findings indicate that the combined loss of 1p and 7p may play a critical role in the tumorigenesis of de novo, aggressive meningiomas.
除了辐射诱导的肿瘤,已知在特定时间段内发展的良性脑膜瘤极为罕见。我们对两例影像学定义的新发、散发性脑膜瘤进行了基因特征分析,一例为 61 岁男性的 5 厘米左侧矢状旁肿瘤,另一例为 53 岁女性的 2.3 厘米右侧大脑镰肿瘤。这两个肿瘤在分别进行与无关疾病相关的 MRI 检查时(手术前分别为 49 个月和 28 个月)均未观察到。两个肿瘤均被完全切除,组织病理学检查显示为 WHO 分级 I 脑膜瘤。两个病例的 MIB-1 染色指数均较高(病例 1 为 5.6%,病例 2 为 9.1%),且免疫组化显示 p53 异常积聚。比较基因组杂交显示,两个肿瘤均存在 1p 和 7p 染色体臂缺失。肿瘤抑制因子 merlin,即 NF2 基因的产物,在两个肿瘤中均未检测到。这两个新发脑膜瘤中共同存在的这些异常可能在脑膜瘤的发病机制和/或快速发展中发挥重要作用。此外,结合以往的研究,我们的发现表明 1p 和 7p 的联合缺失可能在新发侵袭性脑膜瘤的肿瘤发生中发挥关键作用。