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转移性嗜铬细胞瘤/副神经节瘤的临床诊断和预后

邓建华 李汉忠

邓建华, 李汉忠. 转移性嗜铬细胞瘤/副神经节瘤的临床诊断和预后[J]. 协和医学杂志, 2019, 10(6): 654-659. doi: 10.3969/j.issn.1674-9081.2019.06.018
引用本文: 邓建华, 李汉忠. 转移性嗜铬细胞瘤/副神经节瘤的临床诊断和预后[J]. 协和医学杂志, 2019, 10(6): 654-659. doi: 10.3969/j.issn.1674-9081.2019.06.018
Jian-hua DENG, Han-zhong LI. Clinical Diagnosis and Prognosis of Metastatic Pheochromocytoma and Paraganglioma[J]. Medical Journal of Peking Union Medical College Hospital, 2019, 10(6): 654-659. doi: 10.3969/j.issn.1674-9081.2019.06.018
Citation: Jian-hua DENG, Han-zhong LI. Clinical Diagnosis and Prognosis of Metastatic Pheochromocytoma and Paraganglioma[J]. Medical Journal of Peking Union Medical College Hospital, 2019, 10(6): 654-659. doi: 10.3969/j.issn.1674-9081.2019.06.018

转移性嗜铬细胞瘤/副神经节瘤的临床诊断和预后

doi: 10.3969/j.issn.1674-9081.2019.06.018
基金项目: 

中国医学科学院医学与健康创新工程项目 2017-I2M-1-001

详细信息
    通讯作者:

    李汉忠 电话:010-69152511, E-mail:lihzhpumch@163.com

  • 中图分类号: R736

Clinical Diagnosis and Prognosis of Metastatic Pheochromocytoma and Paraganglioma

More Information
  • 摘要: 嗜铬细胞瘤/副神经节瘤(pheochromocytoma and paranganglioma, PPGL)患者由于过度分泌儿茶酚胺, 因而有较高的继发性高血压发生率和死亡率。大约1/4的PPGL具有侵袭转移性, 这类不能切除的转移性病灶的治疗方案包括131I-间碘苄胍核素治疗、放疗和化疗, 但此类治疗也只能控制肿瘤生长和抑制激素水平。过去10年, PPGL的临床和分子机制研究均取得了诸多进展, 然而预后判断和治疗仍然是临床实践中最大的挑战。转移性PPGL患者的自然病史往往未知, 其预后也不尽相同, 目前诊断依然依赖于转移灶的存在, 因此通常诊断时已是晚期。有研究对患者的临床和分子特征、导致转移性PPGL的特定信号通路的改变进行了探索, 以期随着核素治疗、放疗联合分子靶向治疗的发展, 提高该病的临床预后。
    利益冲突  无
  • 表  1  确诊并行手术治疗后5年PPGL患者的转移复发风险[11]

    组别 出现新发复发转移的风险[% (95% CI)]
    嗜铬细胞瘤 8(6~11)
    胸腹盆副神经节瘤 18(11~31)
    头颈部副神经节瘤 25(11~57)
    确诊时年龄≥20岁 9(7~12)
    确诊时年龄<20岁 27(15~51)
    非家族遗传/无基因突变 7(5~11)
    家族遗传综合征/基因突变 17(12~24)
    肿瘤最大直径≤5 cm 10(7~14)
    肿瘤最大直径>5 cm 26(6~100)
    总体 10(8~14)
    PPGL:转移性嗜铬细胞瘤/副神经节瘤
    下载: 导出CSV
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出版历程
  • 收稿日期:  2017-03-03
  • 刊出日期:  2019-11-30

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