Cellular and reticular variants of haemangioblastoma revisited: a clinicopathologic study of 88 cases
Cellular and reticular variants of haemangioblastoma revisited: a clinicopathologic study of 88 cases
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DOI：
10.1111/j.1365-2990.2005.00669.x
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发表时间：
2005-12-01
影响因子：
5
通讯作者：
Paulus, W
中科院分区：
文献类型：
--
作者：
Hasselblatt, M;Jeibmann, A;Paulus, W
血管母细胞瘤的组织学变异的存在已经得到了很好的证实，但关于组织学亚型的预后意义的数据尚不清楚。因此，我们在一系列连续的88例中枢神经系统的原发血管母细胞瘤中，描述了与组织学亚型相关的临床因素，即成血管母细胞瘤的细胞型和网状变异型。根据库欣和贝利，10个血管母细胞瘤被归类为“细胞性”。与更常见的网状变异体(n=78)相比，在细胞性血管母细胞瘤中，含有胶质纤维酸性蛋白阳性肿瘤细胞的肿瘤比例(80%比7%)以及Ki67(MIB1)中值增殖指数[4%(四分位：1-8%)比1%(<；1-2%)]显著更高(P<；0.01)。细胞型的复发更为常见[2/8(25%)vs.4/51(8%)]。Kaplan-Meier分析证实细胞变异体的复发概率显著更高(Log-Rank检验P<；0.01)。Cox回归分析不仅证实了von Hippel-Lindau病与肿瘤复发密切相关(P<；0.01)，而且还显示了组织学亚型对复发概率的独立影响(P<；0.05)，而年龄、性别或肿瘤部位没有明显影响。综上所述，这项回顾性研究的结果表明，血管母细胞瘤的组织学分型具有预后意义，并可能有助于识别有复发风险的患者。
The presence of histological variants of haemangioblastoma is well established, but data on the prognostic implications of histological subtyping are missing. We thus characterized clinical factors associated with histological subtypes, that is, of the cellular and reticular variant of haemangioblastoma, in a series of 88 consecutive primary haemangioblastomas of the central nervous system. Ten haemangioblastomas were classified as 'cellular' according to Cushing and Bailey. As compared to the more common 'reticular' variant (n = 78), the proportion of tumours containing glial fibrillary acidic protein-positive tumour cells (80% vs. 7%), as well as median Ki67 (MIB1) proliferation indices [4% (quartiles: 1-8%) vs. < 1% (< 1-2%)], was significantly higher in cellular haemangioblastomas (P < 0.01). Recurrences were more frequent in the cellular variant [2/8 (25%) vs. 4/51 (8%)]. Kaplan-Meier analysis confirmed a significantly higher probability of recurrence in the cellular variant (Log-Rank test P < 0.01). Cox regression analysis not only confirmed the well established association of von Hippel-Lindau disease with tumour recurrence (P < 0.01), but also revealed an independent effect of histological subtype on the probability of recurrence (P < 0.05), whereas no significant influence of age, sex or tumour location was observed. To conclude, the results from this retrospective study suggest that histological subtyping of haemangioblastomas has prognostic implications and might contribute to identify patients at risk for recurrence.