ショック，急性心不全，カテコラミン心筋症で発症し救命し得た異所性褐色細胞腫の1例

A Rescued Case of Extra-Adrenal Pheochromocytoma Complicated by Cardiogenic Shock,Acute Heart Failure,and Catecholamine Cardiomyopathy

要旨　患者は55歳，女性．2009年6月，娘の結婚式に出席中，背部痛が出現し増悪するため救急車で受診した．来院時，血圧91/70mmHg，心拍数98/分．心電図にてⅡ，Ⅲ，_aV_FでのST上昇，胸部X線にて右上肺野の浸潤影，心エコーにて心基部以外の全周性広範囲のakinesisを認めEF 18%であった．腹部CTにて肝門部に径6cm大の血流に富む内部不均一な腫瘤を認めた．入院直後より胸部浸潤影の急激な増悪に伴い，低血圧，ショック状態となり，ICUにて挿管．その後血行動態維持のためカテコラミン，バソプレシンなどが不可欠であり，退室までに2週間を要した．異所性褐色細胞腫として第38病日手術施行．組織学的に傍神経節腫と確定診断され第46病日徒歩退院した．

　A 55-year-old woman was admitted to our hospital with worsening back pain after her daughter's wedding. Physical examination on admission demonstrated a heart rate of 98/min and blood pressure of 91/70mmHg. ECG showed ST segment elevation in leadsⅡ, Ⅲ, and _aV_F. Chest X-ray demonstrated infiltrative shadows in the right upper lobe, and echocardiography demonstrated extensive akinesis of the anterolateral, apical, and diaphragmatic segments with a global ejection fraction of 18%. Abdominal CT demonstrated a non-uniform mass measuring 6cm in diameter with rich blood flow in the porta hepatis. Worsening pulmonary shadow just after admission caused hypotension and shock, which required emergency mechanical ventilation in the intensive care unit. The control of hemodynamics was poor and systolic blood pressure was 80-90mmHg even with cathecholamine and vasopressin;PaO₂ was 90-100 Torr following diuretics, afterload-reducing medication, and mechanical ventilation. After two weeks of intensive care therapy, surgery was performed on day 38 under a pathological diagnosis of paraganglioma. The patient was ambulatory when discharged home on day 46.