Farrugia F A, Martikos G, Tzanetis P, Charalampopoulos A, Misiakos E, Zavras N, Sotiropoulos D
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Endocr Regul. 2017 Jul 1;51(3):168-181. doi: 10.1515/enr-2017-0018.
We conducted an extensive review of the literature and tried to cite the most recent recommendations concerning the pheochromocytoma (PHEO).
Pub Med and Google Scholar databases were searched systematically for studies concerning pheochromocytomas (intra-adrenal paragangliomas) from 1980 until 2016. Bibliographies were searched to find additional articles.
More than four times elevation of plasma fractionated metanephrines or elevated 24-h urinary fractionated metanephrines are keys to diagnosing pheochromocytoma. If the results are equivocal then we perform the clonidine test. If we have not done it already, we preferably do a CT scan and/or an MRI scan. The patient needs pre-treatment with α1-blockers at least 10-14 days before operation. Alternatives or sometimes adjuncts are Calcium Channels Blockers and/or β-Blockers. Several familial syndromes are associated with PHEO and genetic testing should be considered.
The biggest problem for pheochromocytoma is to suspect it in the first place. Elevated metanephrines establish the diagnosis. With the proper preoperative preparation the risks during operation and the postoperative period are minimal. If there is a risk of the hereditable mutation, it is strongly suggested that all the patients with pheochromocytoma need clinical genetic testing.
我们对文献进行了广泛综述，并试图引用有关嗜铬细胞瘤（PHEO）的最新建议。
系统检索了1980年至2016年期间PubMed和谷歌学术数据库中关于嗜铬细胞瘤（肾上腺内副神经节瘤）的研究。通过检索参考文献以查找其他文章。
血浆去甲肾上腺素分数超过四倍升高或24小时尿去甲肾上腺素分数升高是诊断嗜铬细胞瘤的关键。如果结果不明确，则进行可乐定试验。如果尚未进行，最好进行CT扫描和/或MRI扫描。患者在手术前至少10 - 14天需要用α1阻滞剂进行预处理。替代药物或有时作为辅助药物的是钙通道阻滞剂和/或β阻滞剂。几种家族性综合征与嗜铬细胞瘤有关，应考虑进行基因检测。
嗜铬细胞瘤最大的问题首先是要怀疑它。去甲肾上腺素升高可确诊。通过适当的术前准备，手术中和术后的风险最小。如果存在遗传性突变风险，强烈建议所有嗜铬细胞瘤患者进行临床基因检测。
