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球旁细胞瘤:临床及免疫组化特征

Juxtaglomerular cell tumor: Clinical and immunohistochemical features.

作者信息

Wang Fen, Shi Chuan, Cui Yunying, Li Chunyan, Tong Anli

机构信息

Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.

出版信息

J Clin Hypertens (Greenwich). 2017 Aug;19(8):807-812. doi: 10.1111/jch.12997. Epub 2017 Mar 20.

Abstract

Juxtaglomerular cell tumor (JGCT) is a rare tumor, with approximately 100 cases reported in the literature. The authors respectively studied the clinical data of 11 patients diagnosed with JGCT in Peking Union Medical College Hospital from 2004 to 2014, and investigated the immunohistochemical profiles in 10 tumors. Nine of the 11 patients were diagnosed before the age of 40 years. Hypertension was present in all patients, while hypokalemia occurred in seven of 11 patients. Computed tomography detected JGCTs with a sensitivity of 100%. Immunoreactivities for CD34 and vascular endothelial growth factor were observed in most tumor specimens, suggesting that JGCTs express a variety of vessel-related immunohistochemical markers, although JGCTs are considered a tumor without abundant blood supply. Nuclear accumulation of cyclin D1 was common in JGCTs. Results from immunohistochemistry were negative for BRAF, HER2, and TFE3, suggesting that BRAF, HER2, and TFE3 genes might not play a part in tumorigenesis in JGCTs.

摘要

肾素瘤是一种罕见肿瘤,文献报道约100例。作者分别研究了2004年至2014年在北京协和医院诊断为肾素瘤的11例患者的临床资料,并对其中10例肿瘤进行了免疫组化分析。11例患者中有9例在40岁之前被诊断。所有患者均有高血压,11例患者中有7例出现低钾血症。计算机断层扫描检测肾素瘤的灵敏度为100%。在大多数肿瘤标本中观察到CD34和血管内皮生长因子的免疫反应性,提示肾素瘤表达多种与血管相关的免疫组化标志物,尽管肾素瘤被认为是一种血供不丰富的肿瘤。细胞周期蛋白D1在肾素瘤中常见核内积聚。免疫组化结果显示BRAF、HER2和TFE3均为阴性,提示BRAF、HER2和TFE3基因可能在肾素瘤的肿瘤发生中不起作用。

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