无高血压性嗜铬细胞瘤的临床及病理特点

The clinical and pathological characteristics of normotensive pheochromocytomas

目的探讨无高血压性嗜铬细胞瘤的临床及病理特点。方法回顾南京大学医学院附属鼓楼医院2004年至2013年经病理确诊的97例散发性肾上腺嗜铬细胞瘤病例,其中嗜铬细胞瘤合并高血压64例,无高血压性嗜铬细胞瘤33例,分析其临床表现、血流动力学、代谢指标、激素水平、影像和病理特征。另设原发性高血压95例为对照组。结果无高血压性嗜铬细胞瘤组表现为临床三联征比例低,无明显代谢紊乱,尿儿茶酚胺水平低于嗜铬细胞瘤合并高血压组,但高于原发性高血压组；嗜铬细胞瘤合并高血压组肿瘤重量与24 h尿去甲肾上腺素呈正相关（Y=1.376＋0.653X,R2=0.118,P=0.028）,而无高血压性嗜铬细胞瘤组无此相关性；无高血压性嗜铬细胞瘤组肿瘤直径与组织苯乙醇胺-N-甲基转移酶（ PNMT）蛋白免疫组化染色定性呈负相关（Y=0.940-0.356X,R2=0.494,P=0.005）,而嗜铬细胞瘤合并高血压组无此相关性,提示在临床诊断上无高血压性嗜铬细胞瘤易被漏诊。结论无高血压性嗜铬细胞瘤患者有不同的临床、生化和病理表型,其表型差异与肿瘤发生、发展过程中儿茶酚胺途径的相关产物的表达水平密切相关。

Objective To investigate the clinical and pathological characteristics of normotensive pheochromocytomas （ NP） . Methods This retrospective study included 97 patients with a pathological diagnosis of pheochromocytoma at the Drum Tower Hospital Affiliated to Nanjing University Medical School during January 2004 to December 2013. All available clinical, biochemical, and radiological records were reviewed in these patients who were then categorized into hypertensive pheochromocytomas （HP） （n=64） and NP （n=33） groups. 97 cases of Adrenal Gland Scale Score of pheochromocytoma were examined, including tissue microscopic pathology assessment, ki67 and phenylethanolamine-N-methyltransferase （ PNMT ） immunohistochemistry and catecholamine type. Biochemical examinations of 95 subjects with primary hypertension （ PH） were recorded for comparative study. Results The patients with NP showed lower proportion of clinical triad than HP, inapparent metabolic disorders, and lower urinary catecholamine levels than HP, but showed higher results than primary hypertension. The weight of tumor was positively correlated with 24 hour urinary norepinephrine level in patients with HP（Y=1. 376＋0. 653X,R2=0. 118, P=0. 028）, but not in patients with NP;and the size or diameter of the tumor was negatively correlated with PNMT immunohistochemistry in patients with NP（Y=0. 940-0. 356X, R2=0. 494, P=0. 005）, but not in patients with HP, indicating that NP may be misdiagnosed clinically. Conclusion Patients with NP have distinct clinical, biochemical, and pathological phenotypes; the phenotypic changes are closely related with the expression levels of catecholamine pathway products during the occurrence and development of the tumors.