妊娠合并嗜铬细胞瘤的研究进展

Pheochromocytoma and pregnancy research progress

妊娠合并嗜铬细胞瘤严重影响孕产妇及胎儿生命安全,虽然极其罕见,但可带来灾难性后果。其临床表现多样,与非妊娠患者相似,高血压为其突出表现,由于症状与妊娠相关高血压易混淆,临床上容易被忽视。如果不能确诊,孕产妇及胎儿的死亡率高达50%左右;相反,若早期诊断并采取积极治疗,孕产妇及胎儿的死亡率可分别低于5%、15%。生化检测首选血或尿中儿茶酚胺或代谢产物尿甲氧基肾上腺素,因其具有最高的阴性预测值,唯一可选的定位诊断为MRI,敏感性超过90%。治疗首选腹腔镜肿瘤切除,在妊娠24周内者,应给予与非妊娠者相同药物治疗,术前准备10～14 d后通过腹腔镜肾上腺肿瘤切除。妊娠晚期者,应给予足量药物治疗并常规作好术前准备,直到胎儿成熟并行剖宫产术。经阴道分娩死亡率高,首选剖宫产,如可能剖宫产时一同切除肿瘤。随着诊疗技术的不断进步,孕产妇和胎儿死亡率明显降低、预后明显改善,但最关键最重要的仍是对妊娠高血压怀疑嗜铬细胞瘤患者的早期识别,早期治疗。

A pheochromoeytoma in a pregnant patient is one of the most threatening medical conditions for mother and fetus. Although extraordinarily rare low frequency of all pregnancies, this tumor is notorious for its devastating consequences. As in non-pregnant patients, the signs and symptoms are quite variable but not specific, with hypertension being one of the most prominent signs. Confusion with the much more prevalent forms of pregnancy-related hypertension is the main cause of overlooking the diagnosis. If undiagnosed, maternal and fetal mortality is around 50%. Conversely, early detection and proper treatment during pregnancy decrease the maternal and fetal mortality to〈5 and 15% respect!vely. For the biochemical diagnosis, plasma or urinary metanephrines or its metabolites in urine metanephrines are the tests of first choice since they have a nearly maximal negative predictive value. For reliable localization, only magnetic resonance imaging is suitable, with a sensitivity of more than 90%.When the tumor is diagnosed in the first 24 weeks of gestation, it should be removed by laparoseopic adrenalectomy after 10-14 days of medical preparation with the same drugs as in non-pregnant patients. If the tumor is diagnosed in the third trimester, the patient should be managed until the fetus is viable using the same drug regimen as for regular surgical preparation. Cesarean section with tumor removal in the same session or at a later stage is then preferred since vaginal delivery is possibly associated with higher mortality. Despite all technical diagnostic and therapeutic progress over the last decades, the key factor for further reduction of maternal and fetal mortality is early awareness and recognition of the potential presence of a pheochromocytoma in a pregnant patient with hypertension.