桥小脑角髓母细胞瘤的诊治分析
DOI:
CSTR:
作者:
作者单位:

作者简介:

通讯作者:

中图分类号:

基金项目:


Diagnosis and treatment of cerebellopontine angel medulloblastoma
Author:
Affiliation:

Fund Project:

  • 摘要
  • |
  • 图/表
  • |
  • 访问统计
  • |
  • 参考文献
  • |
  • 相似文献
  • |
  • 引证文献
  • |
  • 资源附件
  • |
  • 文章评论
    摘要:

    目的:探讨桥小脑角(cerebellopontine angle,CPA)髓母细胞瘤的临床、影像及病理相关特点。方法:回顾性分析重庆医科大学附属第一医院近年收治的8例CPA髓母细胞瘤的临床表现、影像学特征、病理分型及手术疗效等资料,并对肿瘤进行分子分型鉴定。结果:本组年龄分布在7~52岁。常见症状为颅内压增高、小脑征、听力下降、面瘫及外展神经麻痹。MRI表现为CPA实性病灶,轻到中度强化,伴瘤周水肿、囊性变、脑膜尾征,CT未见岩骨及内听道破坏及增生。其中6例行全切除,2例行部分切除。术后病理5例经典型,2例促结缔组织增生型,1例间变型。分子鉴定5例为WNT亚型,3例为SHH亚型。术后7例症状体征即刻得到改善,1例维持术前状态。本组随访时间为5~34个月,期间5例呈肿瘤无进展生存状态,3例出现复发或者进展。结论:CPA髓母细胞瘤缺乏特殊的临床表现和影像学特点,在CPA病变的鉴别诊断中应加以考虑。最常见的病理类型和分子亚型分别是经典型和WNT亚型。显微手术是阻止神经功能进一步恶化的有效方法。

    Abstract:

    Objective:To analyze the clinical,imaging and pathological characteristics of cerebellopontine angel(CPA) medulloblas-toma. Methods:Clinical manifestations,imaging characteristics,pathological types and surgical outcomes of eight patients with CPA medulloblastoma were retrospectively analyzed,and their tumors were underwent the molecular grouping and assessment. Results:Patients had an age from 7 to 52 years old,with the most frequent symptoms of intracranial pressure,cerebellar signs,decreased hearing,facial paralysis and abducent nerve paralysis. MRI demonstrated a solid CPA lesion,with mild to moderate enhancement,accompanying with peritumoral oedema,cystic change and dural tail sign,while CT showed no damage and hyperplasia of petrous bone and internal auditory canal intact. Among eight patients,six patients underwent total resection and the other two patients had partial excision. Postoperative pathological examination confirmed five cases of classic type,two cases of desmoplastic type and one case of anaplastic type. Further molecular analysis showed that five cases were WNT subtypes and three cases were SHH subtypes. After the surgery,symptoms and signs of seven patients immediately improved,and condition of one patient remained as the preopera-tive one. Follow-up was ranged from 5-34 months,during which five cases kept symptom free and three cases recurred/progressed. Conclusion:CPA medulloblastoma is rare and lacks special clinical manifestations and imaging features,which needs more attention on differentiating and diagnosing CPA disease. The most common pathological types and molecular subtypes are classical type and WNT subtypes,respectively. Microsurgery is an effective method to prevent the further deterioration of nerve function.

    参考文献
    相似文献
    引证文献
引用本文

吴学东,夏海坚,钟东,杨雅莹,李炯,何大海,史东杰,孙晓川.桥小脑角髓母细胞瘤的诊治分析[J].重庆医科大学学报,2020,45(8):

复制
分享
相关视频

文章指标
  • 点击次数:
  • 下载次数:
  • HTML阅读次数:
  • 引用次数:
历史
  • 收稿日期:
  • 最后修改日期:
  • 录用日期:
  • 在线发布日期: 2020-09-22
  • 出版日期:
文章二维码