Update on Modern Management of Pheochromocytoma and Paraganglioma.
Update on Modern Management of Pheochromocytoma and Paraganglioma.
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DOI：
10.3803/enm.2017.32.2.152
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发表时间：
2017-06
期刊：
影响因子：
--
通讯作者：
Eisenhofer G
中科院分区：
文献类型：
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作者：
Lenders JWM;Eisenhofer G
尽管嗜铬细胞瘤/副神经节瘤的现代诊断方法和治疗方式取得了所有技术进步，但早期考虑这些肿瘤的存在仍然是患者获得最佳结局的关键环节。及时的诊断和适当的治疗可以预防各种潜在的灾难性心血管并发症。现代生化检测应包括提供最佳诊断性能的检测，血浆或尿液中的变肾上腺素和3-甲氧基酪胺的测量。为了尽量减少假阳性试验结果，应特别注意分析前的取样条件。除了通过计算机断层扫描（CT）或磁共振成像进行的解剖成像外，光子发射断层扫描/CT的新的有前途的功能成像模式使用生长抑素类似物，如68 Ga-DOTATATE（68 Ga标记的DOTA（0）-Tyr（3）-奥曲肽），可能会在不久的将来取代123 I-MIBG（碘-123-间碘苄基胍）。由于近一半的嗜铬细胞瘤患者在14种肿瘤易感基因中的一种中存在突变，因此至少应考虑对所有确诊肿瘤的患者进行基因检测和咨询。术后每年通过测量血浆或尿肾上腺素对患者进行随访，应持续至少10年，以及时发现复发或转移性疾病。复发或转移性疾病风险高的患者（副神经节瘤、年轻、多发或大肿瘤、遗传背景）应终身随访。
Despite all technical progress in modern diagnostic methods and treatment modalities of pheochromocytoma/paraganglioma, early consideration of the presence of these tumors remains the pivotal link towards the best possible outcome for patients. A timely diagnosis and proper treatment can prevent the wide variety of potentially catastrophic cardiovascular complications. Modern biochemical testing should include tests that offer the best available diagnostic performance, measurements of metanephrines and 3-methoxytyramine in plasma or urine. To minimize false-positive test results particular attention should be paid to pre-analytical sampling conditions. In addition to anatomical imaging by computed tomography (CT) or magnetic resonance imaging, new promising functional imaging modalities of photon emission tomography/CT using with somatostatin analogues such as 68Ga-DOTATATE (68Ga-labeled DOTA(0)-Tyr(3)-octreotide) will probably replace 123I-MIBG (iodine-123-metaiodobenzylguanidine) in the near future. As nearly half of all pheochromocytoma patients harbor a mutation in one of the 14 tumor susceptibility genes, genetic testing and counseling should at least be considered in all patients with a proven tumor. Post-surgical annual follow-up of patients by measurements of plasma or urinary metanephrines should last for at least 10 years for timely detection of recurrent or metastatic disease. Patients with a high risk for recurrence or metastatic disease (paraganglioma, young age, multiple or large tumors, genetic background) should be followed up lifelong.