脑膜瘤临床病理亚群中Merlin、DAL-1和孕酮受体的表达:175例相关免疫组化研究。

PubMed ID
发表日期 2000年Oct月

原始出处 神经病理学与实验神经学杂志
Journal of neuropathology and experimental neurology
作者 Perry  A  Cai  D X  Scheithauer  B W  Swanson  P E  Lohse  C M  Newsham  I F  Weaver  A  Gutmann  D H 

文献标题 脑膜瘤临床病理亚群中Merlin、DAL-1和孕酮受体的表达:175例相关免疫组化研究。
Merlin, DAL-1, and progesterone receptor expression in clinicopathologic subsets of meningioma: a correlative immunohistochemical study of 175 cases.

文献摘要

脑膜瘤的分子发病机制尚不明确。在所有散发性脑膜瘤中,有30%-80%的病例报告NF2(merlin)表达缺失。最近,我们发现第二种蛋白4.1家族肿瘤抑制因子的表达缺失。DAL-1也很常见。孕酮受体(PR)在生物学上的重要作用也被认为是基于其与肿瘤分级的反向关系。为了更好地确定这些蛋白的致病作用,我们研究了175例完全特征化脑膜瘤的merlin、DAL-1和PR免疫谱,包括非复发性与复发性良性、增殖性与脑侵袭性非典型和间变性亚型。蛋白4.1家族抑癌基因(merlin或DAL-1)的表达缺失几乎是普遍的(92%),联合缺失是常见的(58%)。单独检测,merlin或DAL-1蛋白缺失率分别为74%和76%,5个亚群之间无显著差异。PR免疫反应性与DAL-1的残留表达(p<0.001)和肿瘤分级有关,良性肿瘤51%,非典型肿瘤21%,间变性肿瘤11%染色阳性(p<0.001)。我们认为PR免疫组化可能对脑膜上皮肿瘤有诊断价值。蛋白4.1家族抑癌基因缺失可能是脑膜瘤发病机制中重要的早期事件,而PR表达与良性相关。


The molecular pathogenesis of meningiomas is poorly characterized. Loss of NF2 (merlin) expression has been reported in 30%-80% of all sporadic meningiomas. Recently, we found that loss of expression for a second Protein 4.1-family tumor suppressor. DAL-1, is also common. A biologically important role for progesterone receptor (PR) has also been proposed based on its reported inverse relationship with tumor grade. In order to better define the pathogenetic roles of these proteins, we studied the merlin, DAL-1, and PR immunoprofiles in 175 fully characterized meningiomas, including nonrecurring versus recurring benign, proliferative versus brain invasive atypical and anaplastic subtypes. Loss of expression for either Protein 4.1-family tumor suppressor (merlin or DAL-1) was almost universal (92%), with combined losses being common (58%). Individually, absence of merlin or DAL-1 protein was detected in 74% and 76% respectively, with no significant differences among the 5 subsets. PR immunoreactivity was commonly associated with retained DAL-1 expression (p < 0.001) and with tumor grade, with 51% of benign, 21% of atypical, and 11% of anaplastic tumors staining positive (p < 0.001). We conclude that PR immunohistochemistry may have diagnostic utility in meningothelial neoplasms. Protein 4.1-family tumor suppressor losses are likely important early events in meningioma pathogenesis, whereas PR expression is associated with benignity.


获取全文 10.1093/jnen/59.10.872