Department of General and Endocrine Surgery, CHU Poitiers, 2 Rue de la Miletrie, 86021, Poitiers, France.
Department of General and Endocrine Surgery, CHU Nantes, Nantes, France.
Surg Endosc. 2018 Sep;32(9):3890-3900. doi: 10.1007/s00464-018-6128-x. Epub 2018 Feb 27.
Pheochromocytoma (PHEO) in pregnancy is a life-threatening condition. Its management is challenging with regards to the timing and type of surgery.
A retrospective review of the management of ten patients diagnosed with pheochromocytoma during pregnancy was performed. Data were collected on the initial diagnostic workup, symptoms, treatment, and follow-up.
PHEO was diagnosed in ten patients who were between the 10th and the 29th weeks of pregnancy. Six patients had none to mild symptoms, while four had complications of paroxysmal hypertension. Imaging investigations consisted of MRI, CT scan and ultrasounds. All had urinary metanephrines, measured as part of their workup. Three patients had MEN 2A, one VHL syndrome, one suspected SDH mutation. All patients were treated either with α/β blockers or calcium channel blockers to stabilize their clinical conditions. Seven patients underwent a laparoscopic adrenalectomy before delivery. Three out of these seven patients had a bilateral PHEO and underwent a unilateral adrenalectomy of the larger tumor during pregnancy, followed by a planned cesarean section and a subsequent contralateral adrenalectomy within a few months after delivery. Three patients had emergency surgery for maternal or fetal complications, with C-section followed by concomitant or delayed adrenalectomy. All newborns from the group of planned surgery were healthy, while two out three newborns within the emergency surgery group died shortly after delivery secondary to cardiac and pulmonary complications.
PHEO in pregnancy is a rare condition. Maternal and fetal prognosis improved over the last decades, but still lethal consequences may be present if misdiagnosed or mistreated. A thorough multidisciplinary team approach should be tailored on an individual basis to better manage the pathology. Unilateral adrenalectomy in a pregnant patient with bilateral PHEO may be an option to avoid the risk of adrenal insufficiency after bilateral adrenalectomy.
妊娠期间的嗜铬细胞瘤（pheochromocytoma，PHEO）是一种危及生命的疾病。其管理具有挑战性，涉及手术的时机和类型。
对 10 例妊娠期间诊断为嗜铬细胞瘤的患者的管理进行回顾性分析。收集初始诊断检查、症状、治疗和随访的数据。
PHEO 诊断于妊娠第 10 周至第 29 周的 10 例患者。6 例患者无明显症状，4 例患者出现阵发性高血压并发症。影像学检查包括 MRI、CT 扫描和超声检查。所有患者均进行尿间甲肾上腺素测定，作为其检查的一部分。3 例患者患有 MEN 2A，1 例患有 VHL 综合征，1 例疑似 SDH 突变。所有患者均接受 α/β 阻滞剂或钙通道阻滞剂治疗以稳定其临床状况。7 例患者在分娩前进行了腹腔镜肾上腺切除术。这 7 例患者中有 3 例为双侧 PHEO，在妊娠期间对较大肿瘤进行了单侧肾上腺切除术，随后进行了计划剖宫产，并在分娩后几个月内进行了对侧肾上腺切除术。3 例患者因母婴并发症而行急诊手术，行剖宫产术，随后同时或延迟行肾上腺切除术。计划手术组的所有新生儿均健康，而急诊手术组的 3 例新生儿中有 2 例在分娩后不久因心脏和肺部并发症死亡。
妊娠期间的 PHEO 较为罕见。在过去几十年中，母婴预后有所改善，但如果诊断错误或治疗不当，仍可能导致致命后果。应根据个体情况制定全面的多学科团队方法，以更好地管理该疾病。对于双侧 PHEO 的妊娠患者，单侧肾上腺切除术可能是避免双侧肾上腺切除术后肾上腺功能不全风险的一种选择。
