摘要目的 分析嗜铬细胞瘤危象(pheochromocytoma crisis,PCC)的临床特征,以提高临床医生对本病的认识.方法 回顾性分析重庆医科大学附属第一医院2011年4月至2017年2月期间诊治的123例嗜铬细胞瘤和副神经节瘤(pheochromocytoma and paraganglioma,PPGL)的临床资料,根据有无血流动力学紊乱和终末器官损伤或功能障碍分为危象组和非危象组.比较两组间人口学特点、临床表现、实验室检查、影像学表现、围术期情况及病理资料等的差异.结果 ①危象组16例,其中误诊率31.2％(5例);非危象组107例.②与非危象组相比,危象组头痛、心悸、多汗经典三联征及其他表现更多见,重度高血压、低血压发生率更高,且以阵发性高血压居多,PPGL发作性症状就诊者更多(均P<0.05).③危象组血白细胞、空腹血糖、转氨酶、肌钙蛋白、D-二聚体更高,而估算肾小球滤过率(estimated glomerular filtration rate,eGFR)更低,更多肿瘤位于左侧肾上腺(均P<0.05).④危象组血甲氧基肾上腺素类物质(estimated glomerular filtration rate,MNs)水平、肿瘤最大直径、CT扫描各期CT值、良性肿瘤比例、瘤内出血或坏死情况均高于非危象组,但差异均无统计学意义.⑤危象组多进行择期手术,但术前准备时间、手术方式、术中及术后并发症、死亡率两组间差异均无统计学意义.结论 PCC是一种少见的内分泌急症,其临床表现复杂多样,往往伴有典型三联征,血流动力学更不稳定和终末器官更易受累.虽然依靠生化检查和影像学诊断PCC较为有效,但仍有较高误诊率.一旦明确诊断,应及时启动药物准备,手术切除肿瘤是治疗PCC的关键.

abstractsObjective To analyze the clinical characteristics of pheochromocytoma crisis (PCC). Meth-ods Data of 123 cases of pheochromocytoma and paraganglioma (PPGL) admitted from Apr. 2011 to Feb. 2017 were retrospectively analyzed and they were divided into crisis group and noncrisis group according to the pa-tients with or without haemodynamic instability and end-organ damage. The differences of demographics charac-teristics, presentations, laboratory tests, imaging findings, perioperative clinical conditions and pathological fea-tures were compared between the two groups. Results ①16 cases were enrolled into crisis group, among whom 5 were misdiagnosed, while 107 cases were enrolled into noncrisis group. ②Compared with noncrisis group, the incidence of headache, palpitation, sweating, the classic triad, other presentations of PPGL, severe hypertension and hypotension were higher, and more patients had paroxysmal hypertension and admitted to our hospital for paroxysmal presentations in crisis group (P<0.05). ③Leukocyte, fasting blood glucose, liver transaminases, tro-ponin and D-dimmer were higher, while estimated glomerular filtration rate(eGFR) was lower, more tumors locat-ed in the left of adrenal in crisis group(P<0.05). ④ Patients in crisis group had higher plasma free metanephrines (MNs), larger maximal tumor diameter, higher enhanced CT value in each period, more benign tumors and hem-orrhage or necrosis in the tumors, but all the differences were not significant when compared with the noncrisis group. ⑤Patients in crisis group were more likely to undergo elective surgery. However, there was no difference in the preoperative time of α-blockade, type of surgery, intraoperative and postoperative complications, mortality a-mong the two groups. Conclusions PCC is a rare endocrinological emergency with a highly variable manifesta-tions, which commonly presents with typical triad, with higher incidence of hemodynamic instability and end-organ damage. Although biochemical and imaging examinations are relatively effective in the diagnosis of PCC, the mis-diagnosis remains inappropriate high. Once the diagnosis is established, clinicians should timely start drug prepa-ration while surgical resection is the key to the treatment of PCC.