桥小脑角区原发髓母细胞瘤的病理和分子亚型特点的研究

发布时间：2018-11-25 18:29

【摘要】：目的:桥小脑角区的原发髓母细胞瘤临床少见,目前国外文献报道36例。该病和好发于该区的其他常见病变相比,无特征性的临床和影像学表现,术前诊断较困难。此外该区肿瘤来源多样,其病理学和分子生物学特点认识不清,给该病预后的判断也带来很大困难。本研究旨在通过对CPA区原发髓母细胞瘤的病理学和分子亚型特点进行研究,得到一种有助于判断其预后的方法。方法:回顾性分析本院从2006年1月至2012年6月的11例CPA区原发髓母细胞瘤患者的临床资料并进行随访。对收集到的10例肿瘤标本进行HE和Reticulin染色明确其组织病理学分类,对Gab-1、β-catenin、Flamin A及Yap-1四种抗体进行免疫组织化学染色明确其分子亚型。结合上述资料,进行卡方检验和K-M生存分析。结果:10例CPA区原发髓母细胞瘤标本中,经典型髓母细胞瘤经典型6例(6/10),DN型3例(3/10),大细胞/间变型1例(1/10)。SHH型8例(8/10),WNT型和Non-SHH/WNT型各1例(1/10)。不同年龄组(p=0.007,0.05)、病理类型(p=0.01,0.05)及分子亚型(p=0.007,0.05)的患者之间生存预后显著不同,患者性别、手术切除程度、有无硬膜累及对生存预后无显著影响。结论:CPA区原发髓母细胞瘤临床较少见,其术前诊断困难。该区髓母细胞瘤全切率较低,但术后经系统放化疗的手术全切和近全切患者在生存预后方面无统计学差异。该部位的病理类型相对其他常见部位髓母细胞瘤无明显差别,但SHH亚型所占比例高,提示CPA原发髓母细胞瘤整体预后相对较好。针对不同年龄组的患者进行病理类型和分子亚型的判定,对于个体化预测患者的预后风险具有非常重要的作用。
[Abstract]:Objective: primary medulloblastoma in cerebellopontine angle area is rare. Compared with other common lesions in this area, it is difficult to diagnose the disease before operation because of no characteristic clinical and imaging manifestations. In addition, it is difficult to judge the prognosis of the disease because of the variety of tumor sources and the unclear understanding of its pathological and molecular biological characteristics. The aim of this study was to study the pathological and molecular subtypes of primary medulloblastoma in CPA region and to obtain a method to determine its prognosis. Methods: the clinical data of 11 patients with primary medulloblastoma in CPA area from January 2006 to June 2012 were retrospectively analyzed and followed up. The histopathological classification was determined by HE and Reticulin staining, and the molecular subtypes of Gab-1, 尾-catenin,Flamin A and Yap-1 antibodies were determined by immunohistochemical staining. Combined with the above data, chi-square test and K-M survival analysis were carried out. Results: among the 10 cases of primary medulloblastoma in CPA region, 6 cases were classic type (6 / 10), DN type, 3 cases were 6 / 10), DN type), 1 case was large cell / intervariant type (1 / 10). SHH type, 8 cases (8 / 10%). One case of WNT type and one case of Non-SHH/WNT type (1 / 10). The survival prognosis of patients with different age groups (p0. 007 / 0.05), pathological types (p0. 01 / 0. 05) and molecular subtypes (p0. 007 / 0. 05) were significantly different. There was no significant effect on survival and prognosis with or without dural involvement. Conclusion: primary medulloblastoma in CPA area is rare and difficult to diagnose before operation. The total removal rate of medulloblastoma in this area was low, but there was no significant difference in survival and prognosis between patients undergoing systemic radiotherapy and chemotherapy. There was no significant difference between the pathological types and other common sites of medulloblastoma, but the proportion of SHH subtypes was high, suggesting that the overall prognosis of CPA primary medulloblastoma was relatively good. The classification of pathological types and molecular subtypes for patients with different age groups is very important for individualized prognostic risk prediction.
【学位授予单位】：首都医科大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R739.41

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