血浆游离甲氧基肾上腺素类物质正常的嗜铬细胞瘤/副神经节瘤患者的临床特点分析

Clinical characteristics of pheochromocytoma/paraganglioma patients with normal plasma free metanephrines

目的分析嗜铬细胞瘤/副神经节瘤(PPGL)患者中血浆游离甲氧基肾上腺素类物质(MNs)正常者的临床特点,为该类患者的诊疗提出建议。方法回顾性分析2019年1月-2021年4月在解放军总医院第一医学中心就诊的99例PPGL患者,其中98例经病理证实为PPGL,1例为临床诊断希佩尔-林道综合征(VHL综合征)。所有患者均检测血浆游离MNs,按照MNs水平分为MNs正常的PPGL患者(MNs正常组,n=10)与MNs升高的PPGL患者(MNs升高组,n=89),比较两组患者临床表现、影像学特点、术前准备、术中血压波动等方面的差异。结果MNs正常组患者典型三联征发生率与MNs升高组比较差异无统计学意义(P>0.05),但恶心、呕吐的发生率稍高于MNs升高组(40.0%vs.13.5%,P=0.031)。MNs正常组腹膜后副神经节瘤比例明显高于MNs升高组(60.0%vs.20.2%,P=0.027),具有PPGL典型影像学表现(70.0%vs.92.1%,P=0.028)及肿瘤坏死(40.0%vs.71.9%,P=0.039)的比例明显低于MNs升高组。10例血MNs正常的PPGL患者中,2例尿甲氧基肾上腺素(MN)或甲氧基酪胺(3-MT)轻度升高,8例肿瘤最大直径≥3 cm,7例CT或MRI提示肿瘤具有较为典型的PPGL影像学特征,6例患者功能影像学检查阳性。结论血MNs正常不能作为排除PPGL的充分依据;对于可疑PPGL的患者,如血MNs正常,建议加测尿MNs及3-MT等指标。功能影像学检查对PPGL的诊断具有重要价值。

Objective To analyze the clinical characteristics of pheochromocytoma/paraganglioma(PPGL)patients with normal level of plasma free metanephrines(MNs),and offer a proposal for the diagnosis and treatment of such patients.Methods A retrospective study was conducted of 99 patients hospitalized from January 2019 to April 2021 and diagnosed as PPGL,of which 98 patients were pathologically confirmed as PPGL and 1 patient was clinically diagnosed as Von Hippel-Lindau(VHL)syndrome.Plasma free MNs were detected,and according to the MNs level,all the patients were divided into PPGL with normal MNs group(n=10)and PPGL with elevated MNs group(n=89).The differences were contrasted and analyzed between the two groups in clinical manifestation,imaging characteristics,preoperative preparation and intraoperative blood pressure fluctuation.Results No significant difference was detected in the incidence of typical triad between the two groups,but the incidence of nausea and vomiting in PPGL with normal MNs group was higher(40.0%vs.13.5%,P=0.031).The proportion of retroperitoneal paraganglioma was obviously higher in PPGL with normal MNs group than in PPGL with elevated MNs group(60.0%vs.20.2%,P=0.027),and the proportion of patients possessing typical PPGL imaging manifestations(70.0%vs.92.1%,P=0.028)and tumor necrosis(40.0%vs.71.9%,P=0.039)was lower in PPGL with normal MNs group than in PPGL with elevated MNs group.Among 10 patients in PPGL with normal MNs group,2 cases had slightly increased urinary metanephrine(MN)or 3-MT,and the maximum diameter of tumor in 8 cases was≥3 cm.CT or MRI showed that tumors with typical PPGL imaging features in 7 cases,and functional imaging was positive in 6 cases.Conclusions Normal plasma MNs can not be used as sufficient exclusion of PPGL diagnosis.For patients suspected with PPGL,when normal MNs are obtained,it is recommended to measure extra indexes such as 24 h urine MNs and 3-MT.In addition,functional imaging is of great value in this condition.