目的 提高对嗜铬细胞瘤/副神经节瘤危象认识,加强对嗜铬细胞瘤/副神经节瘤危象诊疗意识.方法 回顾性研究2012年6月至2018年4月本院诊断为嗜铬细胞瘤/副神经节瘤危象患者10例,男性5例(50.0％),女性5例(50.0％),平均年龄(39.10±16.11)岁,其中6例经病理诊断确诊.分析患者的一般情况,嗜铬细胞瘤诊断、临床表现、疾病治疗及预后.结果 10例嗜铬细胞瘤/副神经节瘤患者中5例(50.0％)有慢性高血压病史,2例(20.0％)患者有糖尿病,1例(10.0％)患者有冠心病史.临床症状以恶心、呕吐、气促、胸闷、大汗淋漓最常见.所有患者均以高血压病危象,急性左心衰竭起病,大部分患者病程中出现一过性伴多器官功能障碍综合征或脓毒症,6例(60％)患者接受择期外科手术切除.经治疗后9例(90％)好转出院,患者出院前各器官功能均恢复正常,1例(10％)因反复心衰发作,造成持续肝肾功能、凝血功能障碍,伴真菌感染,死于呼吸循环衰竭.结论 临床上不明原因的高血压危象、休克、左心衰竭、多器官功能障碍综合征、乳酸酸中毒,应考虑嗜铬细胞瘤/副神经节瘤危象可能;早期诊断,尽早外科手术治疗,是减少院内死亡的重要因素.%Objective To strengthen the awareness of the risk of pheochromocytoma/paraganglioma, and improve its diagnosis and treatment outcomes. Methods Retrospective study was performed on 10 patients with pheochromocytoma/paraganglioma between June, 2012 and April, 2018, including 5 cases of male (50.0％) and 5 cases of female (50.0％). The average age was (39.10±16.11) and six cases were pathologically diagnosed of paraganglioma.The patient's general condition, diagnosis outcomes, clinical manifestations, treatment and prognosis were analyzed. Results Among 10 cases of pheochromocytoma/paraganglioma patients, 5 cases (50.0％) had chronic hypertension, 2 cases (20.0％) had diabetes, and 1 case (10.0％) had the history of coronary heart disease. Clinical symptoms were commonly known as nausea, vomiting, shortness of breath, chest oppression and sweating. All patients were diagnosed of hypertensive crisis and acute left heart failure. Most patients developed transient multiple organ dysfunction syndrome or sepsis, including 6 cases (60％) undergoing elective surgical resection. The condition of 9 cases (90％) improved after treatment, with 1 dead case (10％) of respiratory and circulatory failure caused by recurrent heart failure, and dysfunction of liver, kidney and blood coagulation combined with fungal infection. Conclusion The unknown causes of hypertension, shock, left heart failure, multiple organ dysfunction syndrome and lactic acidosis should be considered the crisis of pheochromocytoma/paraganglioma. Early diagnosis and surgical treatment is an important factor to reduce nosocomial mortality.
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