垂体母细胞瘤的临床影像及预后分析  

Clinical Imaging and Prognosis Analysis of Pituitary Blastoma

作　　者：谢珊珊[1] 宋承汝[1] 张勇[1] 李振乾[2] 程敬亮[1] XIE Shan-shan;SONG Cheng-ru;ZHANG Yong;LI Zhen-qian;CHENG Jing-liang(Department of MRI,the First Affiliated Hospital of Zhengzhou University Zhengzhou 450000,Henan Province,China;Department of Pathology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450000,Henan Province,China)

机构地区：[1]郑州大学第一附属医院磁共振科 [2]郑州大学第一附属医院病理科,河南郑州450000

出　　处：《中国CT和MRI杂志》2024年第7期21-24,共4页Chinese Journal of CT and MRI

基　　金：河南省国际科技合作项目(232102521023)。

摘　　要：目的总结PitB患者的临床、影像学特征和预后。方法分析本院1例PitB患儿资料,并以“pituitary blastoma”或“垂体母细胞瘤”为检索词检索Pubmed数据库、中国知网及万方数据库相关文献,总结PitB患者的临床和影像学表现并分析预后。结果共收集23例PitB患者,临床最常表现为Cushing综合征,神经性眼病及发育迟缓。影像表现为鞍区及鞍上囊实性、实性或囊性占位,实性部分明显强化。患者5年OS为(58±11)%。全切/近全切、次全切/未切除组患者5年OS分别为(79±13)%、(42±14)%。接受放化疗和未放化疗患者的5年OS分别为(44±13)%、(86±13)%。Logrank检验分析组间5年OS显示肿瘤全切/近全切组高于次全切/未切除组患者(χ^(2)=4.705,P=0.030),而患者是否接受放化疗与生存率无显著相关性(χ^(2)=1.554,P=0.213)。结论PitB最常见的临床表现是Cushing综合征、神经性眼病及发育迟缓,影像上表现为鞍区及鞍上的囊实性、实性或囊性占位。手术切除方式与患者的生存率有关,是否接受放化疗与患者的生存率无显著相关性。Objective To investigate the clinical characteristics,imaging features and prognosis of pituitary blastoma(PitB).Methods The clinical and imaging characteristics of a case of PitB were studied.Medical literature was searched with the keywords of"pituitary blastoma"in PubMed,China National Knowledge Infrastructure and Wanfang database.Combined with the case,the clinical and imaging findings of the PitB children were summarized and the prognosis was analyzed.Results A total of 23 patients with PitB were collected.The most common clinical manifestations were Cushing syndrome,neuro-ophthalmopathy,developmental delay.PitB mainly manifested as a sellar and suprasellar solid,solid-cystic or cystic mass,and the solid part of the tumor showed obvious enhancement.Except for case 22,13 patients survived and 9 died at the last follow-up.The 5-year overall survival(OS)rate was(58±11)%1.The 5-year OS rates of total/near-total resection and subtotal/non resection group were(79±13)%and(42±14)%,respectively.7/23 patients received chemotherapy or chemoradiotherapy.The 5-year OS rates of patients with chemoradiotherapy and without chemoradiotherapy were(44±13)%and(86±13)%,respectively.Log-rank test showed that the 5-year OS of total/near-total resection group was higher than that in subtotal/non-resection group(χ^(2)=4.705,P=0.030),the differences were statistically significant.While chemoradiotherapy had no statistical significance on OS(χ^(2)=1.554,P=0.213).Conclusion The most common clinical manifestations of PitB were Cushing's syndrome,neuro-ophthalmopathy,and developmental delay.Radiologically,PitBs usually manifested as a sellar and suprasellar solid,solid-cystic or cystic mass.Surgical resection extent was related to the OS rate of the patients,while chemoradiotherapy had no statistical significance on OS.

关 键 词：垂体母细胞瘤 影像 DICER1基因 预后 

分 类 号：R445[医药卫生—影像医学与核医学] R44[医药卫生—诊断学]