Department of Neuropathology, National Institute of Neurology and Neurosurgery, Mexico City, DF, Mexico.
J Neurooncol. 2010 Aug;99(1):41-8. doi: 10.1007/s11060-009-0097-9. Epub 2010 Jan 22.
Chordoid meningioma is a rare variant of meningioma with histological features resembling those of chordoma. This tumor has a great risk of recurrence and aggressive growth (WHO grade II). This study was done to document the clinical and pathological features of ten patients with chordoid meningioma who submitted to surgery at the National Institute of Neurology and Neurosurgery in Mexico City. Clinical, histological and immunohistochemical features were examined. The age range was from 30 to 67 years old (mean, 34.2 years). Seven patients were female and three male. The duration of symptoms varied from 3.5 months to 5 years (mean, 14.1 months). No systemic symptoms were noted. The tumor was localized in eight cases in the supratentorial compartments. Histologically, the tumors were characterized by strands and cords of meningothelial cells arranged in a mucinous stroma. Two of the ten tumors showed metaplasic changes, and seven showed brain invasion. Tumor cells demonstrated CK7, EMA and focal S-100 protein and Ep-CAM. Cytokeratin AE1/AE3, GFAP and synaptophysin were negative. The MIB-1 proliferative index was from 6 to 9% (mean 7.8). PCNA Li was 6 to 20% (mean, 14), and microvascular density was 6-16 (mean, 14.5). The mean rate of the MIB-1 labeling index in recurrences was 7.1% versus 6.33% for no tumor recurrence. Chordoid meningioma, World Health Organization grade II, is an uncommon variant of meningioma with a propensity for aggressive behavior and increased likelihood of recurrence. Chordoid meningiomas are predominantly tumors of young adults with a predilection for the supratentorial location. Intraventricular location and absence of systemic manifestations, despite the presence of abundant B-lymphocytes, mast cells and low MIB-1 LI, are some of the interesting findings in the present series that need further study. Hence, a larger number of cases with adequate follow-up data need to be studied further to establish the clinical relevance of this variant.
脊索样脑膜瘤是脑膜瘤的一种罕见变异型，具有类似于脊索瘤的组织学特征。这种肿瘤具有很高的复发和侵袭性生长的风险（WHO 分级 II）。本研究旨在记录 10 例在墨西哥城国家神经病学与神经外科学院接受手术的脊索样脑膜瘤患者的临床和病理特征。检查了临床、组织学和免疫组织化学特征。年龄范围为 30 岁至 67 岁（平均 34.2 岁）。7 例为女性，3 例为男性。症状持续时间从 3.5 个月到 5 年不等（平均 14.1 个月）。未发现全身症状。8 例肿瘤位于幕上腔。组织学上，肿瘤的特征是排列在黏液基质中的脑膜细胞条索和索状结构。10 例肿瘤中有 2 例有化生改变，7 例有脑侵犯。肿瘤细胞表达 CK7、EMA 和局灶性 S-100 蛋白和 Ep-CAM。细胞角蛋白 AE1/AE3、GFAP 和突触素均为阴性。MIB-1 增殖指数为 6%至 9%（平均 7.8%）。PCNA Li 为 6%至 20%（平均 14%），微血管密度为 6-16（平均 14.5）。复发患者的 MIB-1 标记指数平均率为 7.1%，而无肿瘤复发患者的 MIB-1 标记指数平均率为 6.33%。脊索样脑膜瘤，世界卫生组织分级 II，是一种罕见的脑膜瘤变异型，具有侵袭性行为和复发可能性增加的倾向。脊索样脑膜瘤主要发生于年轻成人，好发于幕上部位。脑室部位和尽管存在大量 B 淋巴细胞、肥大细胞和低 MIB-1 LI，但缺乏全身表现，是本系列中的一些有趣发现，需要进一步研究。因此，需要进一步研究更多具有足够随访数据的病例，以确定这种变异型的临床相关性。
