从PUMCH模式的探索与创新看肾上腺肿瘤的百年诊治历史与发展

Centennial history and development of adrenal tumor treatment from exploring and innovating the PUMCH pattern

在肾上腺肿瘤诊治的百年历程中,北京协和医院从探索疾病的病理生理、组织解剖、内分泌生化检查、特异性核素扫描、复杂病例的多学科讨论、手术方式的不断改良、微创手术的广泛应用、分子机制的不断探索、基因诊断等各个方面,为肾上腺肿瘤的诊治摸索了一套北京协和医院(Peking Union Medical College Hospital,PUMCH)模式.继发性高血压危害大,多与肾上腺肿瘤相关.需要排除的易混淆的类似肾上腺肿瘤相关的原因包括甲亢、绝经期症状、特发性潮热病、高肾上腺素能发作、肾血管病、低血糖、体位性心动过速综合征、肥大细胞病和类癌综合征等,其定性定位诊断相对复杂.北京协和医院建院初期,已有许多肾上腺肿瘤患者,通过临床症状和体征观察、生化检验以及尸检病理解剖等,获得了许多珍贵的资料,使之后更多的患者受益,并且建立了庞大的临床资料数据库和组织标本库,为将来进一步的临床研究打下良好基础.本文综述了北京协和医院肾上腺肿瘤诊治历史与国内外相关进展,以期窥斑见豹,使肾上腺外科疾病的诊治水平不断提升到新的台阶,应对未来新的挑战.

In the past century of diagnosing and treating adrenal tumors,Peking Union Medical College Hospital has explored the pathophysiology,tissue anatomy,endocrine,and biochemical examination.Additionally,specific radionuclide scanning,multidisciplinary discussion of complex cases,continuous improvement of surgical methods,wide application of minimally invasive surgery,continuous exploration of molecular mechanism,gene diagnosis,and other aspects of the disease has been studied to explore a set of PUMCH model for diagnosing and treating adrenal tumors.Secondary hypertension is harmful and is often associated with adrenal tumors;thus,we need to exclude the confusing causes related to adrenal tumors,such as hyperthyroidism,menopausal symptoms,idiopathic tidal fever,hyperadrenergic attack,renal vascular disease,hypoglycemia,postural tachycardia syndrome,mast cell disease,and carcinoid syndrome.At the beginning of Peking Union Medical College Hospital,there were many patients with adrenal tumors.By observing clinical symptoms and signs,biochemical tests,autopsy,and pathological anatomy,valuable data were obtained,making patients benefit from the study.Furthermore,huge clinical and tissue specimen databases were established,laying a good foundation for future clinical research.This article reviews the history of adrenal tumor diagnosis and treatment in Peking Union Medical College Hospital and the related progress at home and abroad in taking the diagnosis and treatment of adrenal surgical diseases to a new level and coping with new future challenges.