Pheochromocytoma and Paraganglioma in Pregnancy: a New Era.
Pheochromocytoma and Paraganglioma in Pregnancy: a New Era.
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DOI：
10.1007/s11886-021-01485-4
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发表时间：
2021-05-07
影响因子：
3.7
通讯作者：
Bancos I
中科院分区：
文献类型：
--
作者：
Gruber LM;Young WF Jr;Bancos I
妊娠期嗜铬细胞瘤和副神经节瘤（PPGL）是一种罕见的疾病，对这些患者的治疗充满了不确定性。我们的目的是回顾目前的文献，并讨论这些患者的诊断和管理。近年来，PPGL在妊娠期的结局有所改善。产妇和胎儿不良结局的最大风险是产后PPGL的诊断。α-和β-肾上腺素能阻滞剂耐受性良好，不良结局较少。产前手术与改善母体或胎儿结局无关。对于已知与PPGL相关的生殖系变异的患者，应在受孕前进行生化检测和横断面成像。当妊娠期间诊断出PPGL时，应开始药物治疗。产前手术应保留到特殊情况。高危患者的病例检测可以在怀孕前识别PPGL。
Pheochromocytoma and paraganglioma (PPGL) in pregnancy is a rare entity and management of these patients is fraught with uncertainty. Our objective is to review current literature and discuss diagnosis and management of these patients. Outcomes of PPGL in pregnancy have improved in recent years. The greatest risk for adverse maternal and fetal outcomes is the diagnosis of PPGL after delivery. Alpha- and beta-adrenergic blockade is well tolerated and is associated with less adverse outcomes. Antepartum surgery is not associated with improved maternal or fetal outcomes. Biochemical testing and cross-sectional imaging should be performed prior to conception for patients with a known germline variant associated with PPGL. Medical therapy should be initiated when PPGL is diagnosed in pregnancy. Antepartum surgery should be reserved for special circumstances. Case detection testing in high-risk patients can identify PPGL before pregnancy.
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