Anesthesia Management in Hereditary Pheochromocytoma and Paraganglioma:Updated Insights into Clinical Features and Perioperative Care

遗传性嗜铬细胞瘤和副神经节瘤的麻醉管理:临床特点和围术期新知

Approximately 40% of pheochromocytoma and paraganglioma(PPGL) cases are familial, typically presenting earlier with more complex symptoms. This paper synthesizes literature and guidelines to inform on clinical characteristics and perioperative care for PPGL. Pheochromocytoma in von Hippel-Lindau(VHL) disease exhibits heightened secretion activity without significant perioperative hemodynamic changes. Tumors in multiple endocrine neoplasia type 2(MEN2) have a stronger endocrine function, which may induce hemodynamic fluctuations during surgery. Therefore, pheochromocytoma screening is essential at all stages of MEN2. Neurofibromatosis type 1(NF1) often presents multisystem lesions and can result in difficult airway. Pheochromocytoma should be evaluated when NF1 patients present hypertension. Pheochromocytoma and paraganglioma type 5 may present multiple lesions of pheochromocytoma or paraganglioma. In summary, hereditary PPGLs may present with severe lesions in other systems, beyond tumor function. A multi-disciplinary team(MDT) approach is often invaluable in perioperative management.

约40%的嗜铬细胞瘤和副神经节瘤(pheochromocytoma andparaganglioma,PPGL)属于遗传性疾病,其发病更早,临床表现更为复杂。本文通过回顾遗传性PPGL的相关文献及指南,旨在为临床诊治提供帮助。希佩尔-林道(von Hippel-Lindau,VHL)病中虽然嗜铬细胞瘤的分泌功能更强,但围术期血流动力学无明显变化。2型多发性内分泌腺瘤病(multipleendocrine neoplasias type 2,MEN2)中的嗜铬细胞瘤分泌功能更强,术中血流动力学波动明显。因此,在MEN2的所有阶段均应筛查嗜铬细胞瘤。1型神经纤维瘤(neurofibromatosis type 1,NF1)通常并发多系统病变且可能存在困难气道,当NF1患者出现高血压时应筛查嗜铬细胞瘤。家族性副节瘤综合征可能合并多发的嗜铬细胞瘤或副神经节瘤。总之,遗传性PPGL通常合并其他系统的严重病变,围术期的多学科团队(multidisciplinary team,MDT)合作在临床诊疗中通常更有意义。