1、n病理：右肾上腺嗜铬细胞瘤病理：右肾上腺嗜铬细胞瘤不典型症状不典型症状Eur J Endocrinol.2009,161:355361临床表现临床表现:取决于儿茶酚胺的分泌类型、释放模式取决于儿茶酚胺的分泌类型、释放模式及个体对儿茶酚胺的敏感性。及个体对儿茶酚胺的敏感性。tyrosineL-多巴多巴多巴胺多巴胺去甲肾上腺素（去甲肾上腺素（NE）肾上腺素（肾上腺素（E）Catecholamines3甲氧基去甲肾上腺素（甲氧基去甲肾上腺素（NMN）3甲氧基肾上腺素甲氧基肾上腺素（MN）PNMTDBHCOMTCOMTMetabolites高香草酸（高香草酸（HVA）MAO,COMT香草扁桃酸（香草扁
2、桃酸（VMA）MAOMAOTumor Secretion:Large Pheo:more metabolites (metabolized within tumor before release)Small Pheo:more catecholamines Sporadic Pheo:Norepi Epi Familial Pheo:Epi Norepi Paraganglioma:Norepi Cheodectoma,glomus jugulare:Norepi Gangioneuroma:Norepi Malignant Pheo:Dopamine,HVA Neuroblastoma:Do
3、pamine,HVATH血浆及血浆及(或或)尿液尿液MNMN和和NMNNMNNat clin pract endocrinol metab.2007,3:92-102.Clin Biochem Rev Vol 30 2009 解剖显像解剖显像功能显像功能显像功能显像的意义功能显像的意义：确诊定位并利于鉴别诊断；确诊定位并利于鉴别诊断；检出多发或转移病灶；检出多发或转移病灶；生化指标阳性和生化指标阳性和(或或)可疑而可疑而CT或或MRI未能定位者；未能定位者；复发病灶的定位等复发病灶的定位等J Clin Endocrinol Metab,May 2010,95:20232037The Journ
4、al of Clinical Endocrinology&Metabolism,2010,95:2596-2606European Journal of Clinical Investigation.doi:10.1111/j.1365-2362.2011.02518.PET scans with F-18-fluorodopamine of patients with adrenal heochromocytomas:left panel shows a left adrenal pheochromocytoma(dark arrow)and right panel shows a righ
5、t adrenal pheochromocytoma(dark arrow).Endocrine-Related Cancer.2012,19:8393 Clin Endocrinol Metab.2010;95(3):1328Ann NY Acad Sci,2006,1037:120.World J Surg，2007，31(4)：849-854中华内分泌外科杂志中华内分泌外科杂志2011，5（3）：）：191193.NEJM,2002,346(19):1459-1466.Ann N Y Acad Sci,2006,1073:30-37.Ann N Y Acad Sci,2006,1073:
6、166-176.Am J Surg,2007,194(6):792-796.EGLN1,MAX EGLN1,MAX Journal of Human Hypertension advance online publication,2012;doi:10.1038/jhh.2012.20中华内分泌代谢杂志.20l0，26：629632中华内分泌代谢杂志中华内分泌代谢杂志.2012，28：526-532中华高血压杂志中华高血压杂志.2012,.2012,:794:794796.796.中华高血压杂志中华高血压杂志.2012,8:794796.European Journal of Endocrinology.2011,164:851870.Journal of Clinical Oncology 2009,27:4162-4168.Nature Clinical Practice Endocrinology and Metabolism 2005,3:92-102 Hormone and Metabolic Research 2008,40:329-337中华内分泌代谢杂志中华内分泌代谢杂志,2011,27:778-781,2011,27:778-781嗜铬细胞瘤的诊治流程嗜铬细胞瘤的诊治流程ThanksThanks
