Luo Shengjun, Cui Qingao, Wang Delin
Urology Department, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China.
Front Oncol. 2022 Jun 20;12:908039. doi: 10.3389/fonc.2022.908039. eCollection 2022.
Progressive multiple organ failures still occur in some patients with pheochromocytoma multisystem crisis (PMC) despite α- and β-blockade being used, and emergency adrenalectomy may lead to rapid hemodynamic stabilization and recovery. Therefore, the optimal timing and surgical approach under PMC remain controversial.
A 50-year-old man presented with persistent chest pain accompanied by vomiting and headache. CT showed a right adrenal mass, and plasma catecholamine levels were significantly elevated. Phenoxybenzamine was used, but his symptoms were aggravated. He progressed to acute respiratory distress syndrome (ARDS) and received mechanical ventilation. Reexamination of CT showed pheochromocytoma rupture. Emergency pheochromocytoma resection was performed on the 5th day, and he was discharged on the 21st day. A 46-year-old woman was admitted for intrauterine device removal and received hysteroscopy under intravenous anesthesia. She presented with dyspnea, fluctuating blood pressure, and loss of consciousness 9 h after hysteroscopy surgery. CT showed a left adrenal mass, and plasma catecholamine levels were significantly elevated. Her condition fluctuated and could not meet the preoperative preparation criteria for pheochromocytoma despite adequate doses of α-blockade and β-blockade were taken. Furthermore, her lung condition worsened due to recurrent crises and pulmonary edema. After multidisciplinary discussions, laparoscopic left adrenalectomy with venoarterial extracorporeal membrane oxygenation (VA-ECMO) support was performed on the 28th day, and she was discharged on the 69th day.
Elective surgical resection is the essential therapy for PMC with adequate preoperative medical management. Emergency surgery is recommended for patients who fail to achieve medical stabilization or progressive organ dysfunction within 1 week, especially those with tumor rupture and uncontrolled bleeding. The laparoscopic approach may represent an option even under PMC.
尽管使用了α和β受体阻滞剂，一些嗜铬细胞瘤多系统危象（PMC）患者仍会出现进行性多器官功能衰竭，而急诊肾上腺切除术可能会使血流动力学迅速稳定并恢复。因此，PMC情况下的最佳手术时机和手术方式仍存在争议。
一名50岁男性因持续性胸痛伴呕吐和头痛就诊。CT显示右侧肾上腺肿块，血浆儿茶酚胺水平显著升高。使用了酚苄明，但他的症状加重。他进展为急性呼吸窘迫综合征（ARDS）并接受了机械通气。CT复查显示嗜铬细胞瘤破裂。在第5天进行了急诊嗜铬细胞瘤切除术，他于第21天出院。一名46岁女性因取出宫内节育器入院，在静脉麻醉下接受了宫腔镜检查。宫腔镜手术后9小时，她出现呼吸困难、血压波动和意识丧失。CT显示左侧肾上腺肿块，血浆儿茶酚胺水平显著升高。尽管服用了足够剂量的α受体阻滞剂和β受体阻滞剂，她的病情仍有波动，不符合嗜铬细胞瘤的术前准备标准。此外，由于反复危象和肺水肿，她的肺部情况恶化。经过多学科讨论，在第28天进行了腹腔镜下左侧肾上腺切除术并给予静脉-动脉体外膜肺氧合（VA-ECMO）支持，她于第69天出院。
择期手术切除是PMC的基本治疗方法，术前需进行充分的药物治疗。对于在1周内未能实现药物稳定或出现进行性器官功能障碍的患者，尤其是那些肿瘤破裂和出血无法控制的患者，建议进行急诊手术。即使在PMC情况下，腹腔镜手术也可能是一种选择。
