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53例髓母细胞瘤的临床病理及免疫组化分析,重点关注突触素表达情况。

A clinicopathologic and immunohistochemical analysis of 53 cases of medulloblastoma with emphasis on synaptophysin expression.

作者信息

Coffin C M, Braun J T, Wick M R, Dehner L P

机构信息

Department of Laboratory Medicine, St. Paul-Ramsey Medical Center/Ramsey Clinic, St. Paul, Minnesota.

出版信息

Mod Pathol. 1990 Mar;3(2):164-70.

PMID:2109316
Abstract

Synaptophysin (SYN) has been identified as an integral membrane glycoprotein of presynaptic vesicles in neurons and neuroendocrine cells, and as a marker for medulloblastoma and other neuronal tumors. SYN expression was studied with a monoclonal antibody (MAb) by the immunoperoxidase technique in 53 medulloblastomas. The expression of neuron-specific enolase (NSE), Leu-7 (LEU), S-100 protein (S100), glial fibrillary acidic protein (GFAP), neurofilament protein (NF), vimentin (VIM), cytokeratin (CKER), and desmin (DES) was also assessed with antisera and MAbs. SYN reactivity was present in 94% of tumors, whereas reactivity with other markers of neuronal differentiation was also observed: NSE (100%) and LEU (83%). Regarding the intermediate filament proteins, 38% of the cases were reactive for VIM, 21% for GFAP, and 9% for DES; none expressed NF or CKER. Eight percent were reactive for S100. Among the 53 cases, the male-female ratio was 1:3; 80% of DES-positive tumors occurred in females. The mean age was 10.5 yr, (60% diagnosed in the first decade; peak age incidence between 5 and 10 yr). Five tumors were discovered in patients older than 20 yr of age. Follow-up showed that 40% of patients developed a recurrence and 47% of patients died of tumor. No statistically significant relationship was demonstrated between patterns of immunoreactivity and prognosis. We conclude that SYN is a useful marker for medulloblastomas, indicating that this tumor is a primitive neuronal-neuroblastic neoplasm. However, it is but one of several immunophenotypic markers expressed by the medulloblastoma.

摘要

突触素(SYN)已被确定为神经元和神经内分泌细胞中突触前囊泡的一种整合膜糖蛋白,也是髓母细胞瘤和其他神经元肿瘤的标志物。采用免疫过氧化物酶技术,用单克隆抗体(MAb)对53例髓母细胞瘤进行了SYN表达研究。还用抗血清和单克隆抗体评估了神经元特异性烯醇化酶(NSE)、Leu-7(LEU)、S-100蛋白(S100)、胶质纤维酸性蛋白(GFAP)、神经丝蛋白(NF)、波形蛋白(VIM)、细胞角蛋白(CKER)和结蛋白(DES)的表达。94%的肿瘤有SYN反应性,同时也观察到与其他神经元分化标志物的反应性:NSE(100%)和LEU(83%)。关于中间丝蛋白,38%的病例对VIM有反应,21%对GFAP有反应,9%对DES有反应;均未表达NF或CKER。8%对S100有反应。53例中,男女比例为1:3;DES阳性肿瘤的80%发生在女性。平均年龄为10.5岁(60%在第一个十年确诊;发病高峰年龄在5至10岁之间)。5例肿瘤发现于20岁以上患者。随访显示,40%的患者复发,47%的患者死于肿瘤。免疫反应模式与预后之间未显示出统计学上的显著关系。我们得出结论,SYN是髓母细胞瘤的一种有用标志物,表明该肿瘤是一种原始的神经元-神经母细胞瘤性肿瘤。然而,它只是髓母细胞瘤表达的几种免疫表型标志物之一。

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