Department of Endocrinology and Metabolism, Tianjin Medical University General Hospital, Tianjin, China.
Front Endocrinol (Lausanne). 2020 Sep 29;11:586795. doi: 10.3389/fendo.2020.586795. eCollection 2020.
Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors, characterized by excessive release of catecholamines (CAs), and manifested as the classic triad of headaches, palpitations, profuse sweating, and a variety of other signs and symptoms. The diagnosis of PPGL requires both evidence of excessive release of CAs and anatomical localization of CA-secreting tumor. Surgery is the mainstay of treatment for all patients with PPGL unless contraindicated. However, without proper preparation, the release of excessive CAs, especially during surgery, can result in lethal cardiovascular complications. Herein, we briefly reviewed the pathogenesis of this disease, discussed the current approaches and evidence available for preoperative management, summarizing the results of the latest studies which compared the efficacies of preoperative management with or without α adrenergic-receptor antagonists, aiming to facilitate better understanding of the preoperative management of PPGL for the physicians.
嗜铬细胞瘤和副神经节瘤(PPGL)是罕见的神经内分泌肿瘤,其特征是儿茶酚胺(CA)过度释放,并表现出经典的三联征,即头痛、心悸、多汗,以及各种其他体征和症状。PPGL 的诊断需要同时有 CA 过度释放的证据和 CA 分泌肿瘤的解剖定位。手术是所有 PPGL 患者的主要治疗方法,除非有禁忌症。然而,如果没有适当的准备,CA 的过度释放,尤其是在手术过程中,可能会导致致命的心血管并发症。在此,我们简要回顾了这种疾病的发病机制,讨论了目前用于术前管理的方法和可用证据,总结了最新研究的结果,比较了术前使用和不使用α肾上腺素能受体拮抗剂的管理效果,旨在帮助医生更好地理解 PPGL 的术前管理。