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摘要: 目的 探索和比较肾上腺偶发嗜铬细胞瘤与症状导向型嗜铬细胞瘤患者的临床特征。方法 回顾性分析2017年5月—2023年5月北京大学第一医院收治的227例肾上腺嗜铬细胞瘤患者，收集并比较患者的一般资料、诊断模式、症状特点、肿瘤大小、手术情况等资料。结果 肾上腺偶发嗜铬细胞瘤患者共129例(56.8%)，症状导向型嗜铬细胞瘤患者74例(32.6%)，基因突变患者10例，术后复发患者9例，恶性肿瘤随访和全身评价过程中发现5例。与症状导向型嗜铬细胞瘤患者比较，肾上腺偶发嗜铬细胞瘤患者在典型症状及非特异性症状发生率、高血压发生率、阵发性高血压比例、血压峰值、联合肾脏切除比例方面差异有统计学意义(P < 0.05)。二者在性别、年龄、体重指数(BMI)、肿瘤位置及肿瘤大小方面比较差异无统计学意义(P>0.05)。结论 由于健康体检与影像学技术的普及，嗜铬细胞瘤的诊断模式正逐渐改变，需要提高大众健康意识来改善嗜铬细胞瘤的诊断和治疗。Abstract: Objective To explore and compare the clinical characteristics of incidental adrenal pheochromocytoma and symptom-guided pheochromocytoma patients.Methods A retrospective analysis was conducted on 227 patients with pheochromocytoma treated at Peking University First Hospital from May 2017 to May 2023. Patient demographics, diagnostic patterns, symptom characteristics, tumor sizes, and surgical outcomes were collected and compared.Results Among the 227 cases, there were 129 cases(56.8%) of incidental adrenal pheochromocytoma and 74 cases(32.6%) of symptom-guided pheochromocytoma. There were 10 cases with genetic mutations, 9 cases of postoperative recurrence, and 5 cases detected during follow-up and systemic evaluation with malignant tumors. Statistically significant differences were observed between incidental adrenal pheochromocytoma and symptom-guided pheochromocytoma in terms of the occurrence of typical and non-specific symptoms, the incidence of hypertension, the proportion of paroxysmal hypertension, peak blood pressure values, and the rate of combined renal resection(P < 0.05). There were no statistically significant differences between the two groups in terms of gender, age, BMI, tumor location, and tumor size(P>0.05).Conclusion With the increasing prevalence of health caring and advances in imaging technology, the diagnostic patterns of pheochromocytoma are gradually changing. It is necessary to raise public awareness of health to improve the diagnosis and treatment of pheochromocytoma.
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Key words:
- pheochromocytoma /
- adrenal incidentaloma /
- paroxysmal hypertension /
- catecholamines
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表 1 肾上腺偶发嗜铬细胞瘤发现情况
肾上腺偶发嗜铬细胞瘤发现症状 例(%) 健康体检 91(70.5) 腹痛/腹胀、腰痛 11(8.5) 心肺症状(咳嗽咳痰、胸闷胸痛、发热等)行胸部影像学检查 9(7.0) 随访其他疾病(肺结节、肝血管瘤、胆结石等) 6(4.7) 泌尿系症状及疾病(血尿、蛋白尿、肾移植等) 5(3.9) 评价其他疾病时(甲亢、糖尿病、脑梗等) 4(3.1) 怀疑肿瘤(如乏力等) 2(1.6) 外伤 1(0.8) 
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表 2 临床资料
例(%)，X±S，M(Q1，Q3) 项目 偶发嗜铬细胞瘤(129例) 症状导向型嗜铬细胞瘤(74例) P值 性别 0.353 女 61(47.3) 40(54.1) 男 68(52.7) 34(45.9) 年龄/岁 52.0(39.0，60.0) 49.5(35.0，60.5) 0.398 BMI/(kg/m2) 23.24±3.14 23.98±3.81 0.136 高血压 80(62.0) 68(91.9) < 0.001 高血压类型 < 0.001 阵发性高血压 12(9.3) 10(13.5) 持续性高血压 59(45.7) 32(43.2) 持续性伴阵发性升高 9(7.0) 26(35.1) 病程中最高SBP 150(128，180) 180(159，210) < 0.001 病程中最高DBP 90(76，100) 107(95，120) < 0.001 体位性低血压 2(1.6) 2(2.7) 0.623 儿茶酚胺心肌病 0(0) 5(6.8) 0.006 血浆3-MT/(pmol/L) 80.0(30.4，80.0) 80.0(32.6，80.0) 0.870 血浆MN/(pmol/L) 426.7(132.0，1 614.8) 541.8(141.2，2 762.5) 0.380 血浆NMN/(pmol/L) 4 150.0(1 319.1，12 360.5) 6 084.4(2 370.8，10 486.6) 0.286 3-MT升高倍数 0.44(0.27，0.44) 0.44(0.33，0.52) 0.691 血MN升高倍数 0.96(0.29，3.30) 1.43(0.34，5.53) 0.239 血NMN升高倍数 4.86(1.47，13.72) 7.53(3.00，13.16) 0.270 肿瘤位置 0.051 单发 123(95.3) 71(95.9) 左侧 55(42.6) 37(50.0) 右侧 68(52.7) 34(45.9) 双侧 1(0.8) 3(4.1) 多发 5(3.9) 0(0) 肿瘤单侧多病灶 0(0) 3(4.1) 0.047 肿瘤直径/cm 4.5(3.5，5.8) 4.5(3.5，6.1) 0.955 联合肾脏切除 8(6.2) 0(0) 0.028 注：SBP：收缩压; DBP：舒张压; 3-MT：3-甲氧基酪胺; MN：甲氧基肾上腺素; NMN：甲氧基去甲肾上腺素。
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表 3 临床症状比较
例(%) 临床症状 偶发嗜铬细胞瘤(129例) 症状导向型嗜铬细胞瘤(74例) P值 头痛 13(10.1) 46(62.2) < 0.001 心悸 24(18.6) 39(52.7) < 0.001 大汗 19(14.7) 23(31.1) 0.006 三联征 4(3.1) 15(20.3) < 0.001 头晕 24(18.6) 33(44.6) < 0.001 腰痛/腹痛 14(10.9) 4(5.4) 0.189 腹胀 4(3.1) 1(1.4) 0.655 恶心呕吐 1(0.8) 20(27.0) < 0.001 发热 3(2.3) 3(4.1) 0.670 胸闷 3(2.3) 9(12.2) 0.010 胸痛 3(2.3) 3(4.1) 0.670 气短 4(3.1) 8(10.8) 0.032 晕厥 2(1.6) 1(1.4) 1.000 眼痛 0(0) 1(1.4) 0.365 视物模糊 0(0) 5(6.8) 0.006 面色苍白 1(0.8) 5(6.8) 0.025 面部潮红 2(1.6) 3(4.1) 0.357 四肢冰冷 0(0) 3(4.1) 0.047 肢体麻木 1(0.8) 6(8.1) 0.010 肢体震颤 1(0.8) 5(6.8) 0.025 肢体水肿 0(0) 4(5.4) 0.017 失眠 3(2.3) 0(0) 0.555 惊恐/焦虑 1(0.8) 3(4.1) 0.139 便秘 3(2.3) 5(6.8) 0.144 乏力 9(7.0) 19(25.7) < 0.001 体重减轻 9(7.0) 12(16.2) 0.037 无症状 62(48.1) 6(8.1) < 0.001
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[1] Mete O, Asa SL, Gill AJ, et al. Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas[J]. Endocr Pathol, 2022, 33(1): 90-114. doi: 10.1007/s12022-022-09704-6
[2] Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and Paraganglioma[J]. N Engl J Med, 2019, 381(6): 552-565. doi: 10.1056/NEJMra1806651
[3] Fassnacht M, Arlt W, Bancos I, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors[J]. Eur J Endocrinol, 2016, 175(2): G1-G34. doi: 10.1530/EJE-16-0467
[4] Kopetschke R, Slisko M, Kilisli A, et al. Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma[J]. Eur J Endocrinol, 2009, 161(2): 355-361. doi: 10.1530/EJE-09-0384
[5] Wachtel H, Cerullo I, Bartlett EK, et al. Clinicopathologic characteristics of incidentally identified pheochromocytoma[J]. Ann Surg Oncol, 2015, 22(1): 132-138. doi: 10.1245/s10434-014-3933-x
[6] Rogowski-Lehmann N, Geroula A, Prejbisz A, et al. Missed clinical clues in patients with pheochromocytoma/paraganglioma discovered by imaging[J]. Endocr Connect, 2018, 7(11): 1168-1177. doi: 10.1530/EC-18-0318
[7] Gruber LM, Hartman RP, Thompson GB, et al. Pheochromocytoma Characteristics and Behavior Differ Depending on Methodsof Discovery[J]. J Clin Endocrinol Metab, 2019, 104(5): 1386-1393. doi: 10.1210/jc.2018-01707
[8] Ebbehoj A, Stochholm K, Jacobsen SF, et al. Incidence and Clinical Presentation of Pheochromocytoma and Sympathetic Paraganglioma: A Population-based Study[J]. J Clin Endocrinol Metab, 2021, 106(5): e2251-e2261. doi: 10.1210/clinem/dgaa965
[9] Zhang J, Li M, Pang Y, et al. Genetic Characteristics of Incidental Pheochromocytoma and Paraganglioma[J]. J Clin Endocrinol Metab, 2022, 107(5): e1835-e1842. doi: 10.1210/clinem/dgac058
[10] Lenders J, Kerstens MN, Amar L, et al. Genetics, diagnosis, management and future directions of research of phaeochromocytoma and paraganglioma: a position statement and consensus of the Working Group on Endocrine Hypertension of the European Society of Hypertension[J]. J Hypertens, 2020, 38(8): 1443-1456. doi: 10.1097/HJH.0000000000002438
[11] Amar L, Servais A, Gimenez-Roqueplo AP, et al. Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paraganglioma[J]. J Clin Endocrinol Metab, 2005, 90(4): 2110-2116. doi: 10.1210/jc.2004-1398
[12] Soltani A, Pourian M, Davani BM. Correction to: Does this patient have Pheochromocytoma? a systematic review of clinical signs and symptoms[J]. J Diabetes Metab Disord, 2017, 16: 42. doi: 10.1186/s40200-017-0324-4
[13] Jain A, Baracco R, Kapur G. Pheochromocytoma and paraganglioma-an update on diagnosis, evaluation, and management[J]. Pediatr Nephrol, 2020, 35(4): 581-594. doi: 10.1007/s00467-018-4181-2
[14] Júnior LJV, Kater CE. The Pheochromocytoma/Paraganglioma syndrome: an overview on mechanisms, diagnosis and management[J]. Int Braz J Urol, 2023, 49(3): 307-319. doi: 10.1590/s1677-5538.ibju.2023.0038
[15] Ebbehoj A, Li D, Kaur RJ, et al. Epidemiology of adrenal tumours in Olmsted County, Minnesota, USA: a population-based cohort study[J]. Lancet Diabetes Endocrinol, 2020, 8(11): 894-902. doi: 10.1016/S2213-8587(20)30314-4
[16] Eisenhofer G, Timmers HJ, Lenders JW, et al. Age at diagnosis of pheochromocytoma differs according to catecholamine phenotype and tumor location[J]. J Clin Endocrinol Metab, 2011, 96(2): 375-384. doi: 10.1210/jc.2010-1588
[17] Pappachan JM, Raskauskiene D, Sriraman R, et al. Diagnosis and management of pheochromocytoma: a practical guide to clinicians[J]. Curr Hypertens Rep, 2014, 16(7): 442. doi: 10.1007/s11906-014-0442-z
[18] Pappachan JM, Tun NN, Arunagirinathan G, et al. Pheochromocytomas and Hypertension[J]. Curr Hypertens Rep, 2018, 20(1): 3. doi: 10.1007/s11906-018-0804-z
[19] Pappachan JM, Buch HN. Endocrine Hypertension: A Practical Approach[J]. Adv Exp Med Biol, 2017, 956: 215-237.
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| 引用本文: |
韩文聪, 胡文晖, 陈仕炜, 等. 嗜铬细胞瘤发现方式的临床特征比较分析[J]. 临床泌尿外科杂志, 2023, 38(11): 810-814. doi: 10.13201/j.issn.1001-1420.2023.11.002
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| Citation: |
HAN Wencong, HU Wenhui, CHEN Shiwei, et al. A comparative analysis of clinical characteristics in pheochromocytoma detection[J]. J Clin Urol, 2023, 38(11): 810-814. doi: 10.13201/j.issn.1001-1420.2023.11.002
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