pheochromocytoma multisystem crisisに至った最重症の褐色細胞腫における周術期管理の1症例

Perioperative Management of a Pheochromocytoma Multisystem Crisis

要旨

　今回，静脈-動脈体外式膜型人工肺（veno-arterial extracorporeal membrane oxygenation：V-A ECMO）の使用下で緊急手術を施行したpheochromocytoma multisystem crisis（PMC）を経験した。PMCは褐色細胞腫の中でも多臓器障害を伴う重篤な病態である。褐色細胞腫の治療原則は術前管理後の待機的手術であるが，生命を脅かすような最重症例においては，V-A ECMOサポート下での緊急手術が，唯一の救命手段となる可能性がある。

　Pheochromocytoma multisystem crisis（PMC）is a fatal condition of pheochromocytoma that is characterised by multiple organ failure, severe blood pressure abnormalities, fever＞40℃, and encephalopathy. The treatment for pheochromocytoma includes preoperative management with selective α₁-blockers and fluid management, followed by surgical tumour resection. We recently treated a 68-year-old male with PMC for whom an emergency adrenal tumourectomy was performed under the support of veno-arterial extracorporeal membrane oxygenation（V-A ECMO）at 3 days post-onset. In the most severe life-threatening PMC cases, emergency surgery may be the best life-saving option.