[1]王芬,童安莉,曾正陪.恶性嗜铬细胞瘤/副神经节瘤的治疗[J].国际内分泌代谢杂志,2016,36(03):187-190.[doi:10.3760/cma.j.issn.1673-4157.2016.03.11]
　Wang Fen,Tong Anli,Zeng Zhengpei..Treatment of malignant pheochromocytomas and paragangliomas[J].International Journal of Endocrinology and Metabolism,2016,36(03):187-190.[doi:10.3760/cma.j.issn.1673-4157.2016.03.11]
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恶性嗜铬细胞瘤/副神经节瘤的治疗()
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《国际内分泌代谢杂志》[ISSN:1673-4157/CN:12-1383/R]

卷:
36
期数:
2016年03期
页码:
187-190
栏目:
综述
出版日期:
2016-05-20

文章信息/Info

Title:
Treatment of malignant pheochromocytomas and paragangliomas
作者:
王芬; 童安莉; 曾正陪
100730 北京,中国医学科学院北京协和医院内分泌科,卫生和计划生育委员会内分泌重点实验室
Author(s):
Wang Fen;  Tong Anli;  Zeng Zhengpei.
Department of Endocrinology, Key Laboratory of Endocrinology, National Health and Family Planning Commission, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Science,Beijing 100730,China
关键词:
恶性嗜铬细胞瘤;  恶性副神经节瘤;  治疗
Keywords:
Malignant pheochromocytoma;  Malignant paraganglioma;  Therapy
DOI:
10.3760/cma.j.issn.1673-4157.2016.03.11
摘要:
嗜铬细胞瘤/副神经节瘤是一种少见的神经内分泌肿瘤,其中10%～17%为恶性肿瘤。其死亡率很高,治疗上存在很大难度。治疗方法包括放射性核素治疗、化学治疗、靶向治疗等。131I-MIBG治疗是应用最广泛的治疗方法,能延缓病情进展,提高生存时间,但仅仅对于肾上腺髓质显像阳性的病灶有效; 奥曲肽显像在定位诊断中体现出很大优势,但核素标记的生长抑素类似物治疗因不良反应和经验较少却应用不多,目前相关文献有限; 化学治疗也是一种有效的治疗方法,但治疗时间长,不良反应中等; 苏尼替尼在靶向治疗中显示出应用前景,但仍然需要更多证据支持。
Abstract:
Pheochromocytoma and paraganglioma(PPGL)are rare neuroendocrine tumors. Malignant PPGL, accounting for 10%-17% of all PPGL, has a high mortality rate. And its treatment is challenging. Several therapies are available nowadays including radionuclide therapy, chemotherapy, target therapy, etc. 131MIBG radiotherapy is the most widely applied method which is effective in delaying disease progression and elevating survival time in positive MIBG scintigraphy. Somatostatin receptor scintigraphy is important in detecting metastasis lesions while radiolabeled octreotide derivatives are seldom employed because of side effects and less experience. Chemotherapy is also an effective method with a moderate side effect. But it needs to be employed monthly. Sunitinib seems to be promising, but evidence at present is limited.

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更新日期/Last Update: 2016-05-20