Prete Alessandro, Paragliola Rosa Maria, Salvatori Roberto, Corsello Salvatore Maria
Endocr Pract. 2016 Mar;22(3):357-70. doi: 10.4158/EP151009.RA. Epub 2015 Nov 4.
Catecholamine-secreting tumors (pheochromocytomas and paragangliomas) presenting during pregnancy are extremely rare, but they can be fatal to both mother and fetus. Recent discoveries in the genetic background of these tumors are expected to address an increasing number of at-risk women to prenatal diagnosis.
The literature was reviewed in order to provide clinicians with a practical and updated guide on how to manage this life-threatening condition.
The clinical presentation of catecholamine-secreting tumors can be deceptive and mimic common disorders of pregnancy. Silent catecholamine-secreting tumors can become evident during pregnancy, and hypertension cannot be considered a hallmark for this condition: some women may be normotensive or develop orthostatic hypotension. Biochemical screening includes measurement of plasma free metanephrines or urinary fractioned metanephrines. Measurement of catecholamines, dopamine, and methoxytyramine can provide further information on tumor biology, location, and prognosis. Diagnostic imaging is limited, and medical treatment requires a cautious balance between hemodynamic control and effects on the fetoplacental unit. Several genes have been associated with syndromes including catecholamine-secreting tumors, and positive genetic testing can correlate with tumor behavior. Timing and modalities for tumor removal and delivery, including anesthetic management, depend on gestational age, maternal and fetal wellbeing, control of catecholamine excess, suspicion of multiple or malignant disease, and surgical accessibility to the tumor.
A timely diagnosis and a multidisciplinary approach are the keys to improve pregnancy outcomes in patients with a catecholamine-secreting tumor; each case should be managed in a tertiary referral center.
孕期出现的分泌儿茶酚胺的肿瘤（嗜铬细胞瘤和副神经节瘤）极为罕见，但对母亲和胎儿都可能致命。这些肿瘤遗传背景方面的最新发现有望让越来越多的高危女性接受产前诊断。
对文献进行综述，以便为临床医生提供有关如何处理这种危及生命状况的实用且最新的指南。
分泌儿茶酚胺肿瘤的临床表现可能具有欺骗性，会模仿常见的妊娠疾病。无症状的分泌儿茶酚胺肿瘤在孕期可能会显现出来，高血压不能被视为这种疾病的标志：一些女性可能血压正常或出现体位性低血压。生化筛查包括测定血浆游离甲氧基肾上腺素或尿分馏甲氧基肾上腺素。测定儿茶酚胺、多巴胺和甲氧基酪胺可为肿瘤生物学、位置及预后提供更多信息。诊断性影像学检查受限，药物治疗需要在血流动力学控制与对胎儿 - 胎盘单位的影响之间谨慎权衡。有几个基因与包括分泌儿茶酚胺肿瘤在内的综合征相关，基因检测呈阳性可能与肿瘤行为相关。肿瘤切除和分娩的时机及方式，包括麻醉管理，取决于孕周、母婴健康状况、儿茶酚胺过量的控制情况、对多发或恶性疾病的怀疑以及肿瘤的手术可及性。
及时诊断和多学科方法是改善分泌儿茶酚胺肿瘤患者妊娠结局的关键；每个病例都应在三级转诊中心进行处理。
