Pheochromocytoma and pregnancy: the epitome of high risk.
Pheochromocytoma and pregnancy: the epitome of high risk.
复制标题
嗜铬细胞瘤和妊娠：高风险的缩影。
作者：
D. T. Freier;N. Thompson
背景
过去的审查表明，如果在分娩前未在母亲体内检测到肿瘤，则一半的胎儿和近一半的母亲会死于嗜铬细胞瘤。这种情况的罕见性增加了潜在的危险，因为诊断和治疗的经验不寻常。然而，考虑时诊断很简单，因为怀孕不会改变儿茶酚胺的诊断水平。管理必须个性化并且可以安全地进行。
方法
本文介绍五例妊娠期嗜铬细胞瘤患者的病例。通过尿儿茶酚胺测量进行诊断。定位方法包括腔静脉导管插入术和超声检查。使用α-和β-肾上腺素能阻滞剂控制血压。治疗策略是由内科医生、产科医生、外科医生和麻醉师组成的团队制定的。
结果
五名母亲和三个胎儿全部幸存。两例胎儿死亡均为计划终止妊娠。两名母亲和一名胎儿患有多发性内分泌肿瘤 2B 型综合征。一位母亲有两次复发，其中一次与怀孕有关。
结论
怀孕不会改变尿儿茶酚胺水平来混淆嗜铬细胞瘤的诊断。超声检查和磁共振成像定位对胎儿是安全的。 α-和β-肾上腺素能阻滞剂具有良好的耐受性。当在妊娠晚期首次发现肿瘤时，应尽一切努力挽救正常胎儿。切除的时间最好由团队规划来决定。
BACKGROUND
Past review has shown that one half of the fetuses and nearly that many mothers died of pheochromocytoma if the tumor was not detected in the mother before delivery. The rarity of the occurrence adds to the potential danger because experience with diagnosis and management is unusual. However, the diagnosis is straightforward when considered because pregnancy does not alter diagnostic levels of catecholamines. Management must be individualized and can be done safely.
METHODS
The cases of five patients with pheochromocytomas during pregnancy are presented here. Diagnosis was made by urinary catecholamine measurement. Localization methods included caval catheterization and ultrasonography. Blood pressure control was done with alpha- and beta-adrenergic blockers. Treatment strategy was planned by a team of internists, obstetricians, surgeons, and anesthesiologists.
RESULTS
All five mothers and three fetuses survived. The two fetal deaths were planned terminations. Two mothers and one fetus had multiple endocrine neoplasia type 2B syndrome. One mother had two recurrences, one of which was involved with the pregnancy.
CONCLUSIONS
Pregnancy does not alter urinary catecholamine levels to confuse the diagnosis of pheochromocytoma. Localization by ultrasonography and magnetic resonance imaging is safe to the fetus. alpha- And beta-adrenergic blockers are well tolerated. Every effort should be made to save a normal fetus when the tumor is first discovered during the third trimester. Timing of excision is a decision best done by team planning.