Department of Anesthesiology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medicine Science, Beijing, China.
State Key Laboratory of Complex Severe and Rare Diseases (Peking Union Medical College Hospital), Beijing, China.
Front Endocrinol (Lausanne). 2022 Jun 30;13:905963. doi: 10.3389/fendo.2022.905963. eCollection 2022.
Multiple endocrine neoplasia type 2A (MEN2A) is a rare syndrome that presents as medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. Experience is lacking in the anesthetic management of patients with this syndrome, particularly in those who present with pheochromocytoma receiving nonpheochromocytoma resection. We aimed to share our experience with the anesthetic management of MEN2A patients.
We retrospectively enrolled 24 MEN2A patients who had received different types of surgery at Peking Union Medical College Hospital from January 1, 2015, to December 31, 2021. All the medical records were reviewed and analyzed.
In total, 33 surgeries were performed in 24 MEN2A patients, with 20 surgeries comprising pheochromocytoma resection in 17 patients. Most of these patients who had received pheochromocytoma resection had typical hemodynamic changes during surgery and anesthesia. Regarding the other 13 nonpheochromocytoma resections in 13 patients, 10 were performed in patients without pheochromocytoma, and 3 surgeries were performed with either functional primary (1, bilateral tumor whose patient refused adrenalectomy) or metastatic pheochromocytoma (2, unresectable and malign tumors developed years after bilateral adrenalectomy). Regarding the latter 3 patients, 1 showed hypertension and tachycardia during anesthesia induction, 1 showed tachycardia during surgery and the other showed stability during surgery. Patients who had received pheochromocytoma resection (n=17) required longer postoperative hospital stays than those who had received nonpheochromocytoma resection without pheochromocytoma (n=10) (5.8 ± 1.8 vs. 4.3 ± 1.6; P = 0.031).
Whenever MEN2A patients are diagnosed with pheochromocytoma, surgical resection of the pheochromocytoma remains the primary choice for MEN2A treatment. Nonpheochromocytoma surgeries performed with existing pheochromocytoma could be risky and require full caution and preparation.
多发性内分泌腺瘤病 2A 型（MEN2A）是一种罕见的综合征，表现为甲状腺髓样癌、嗜铬细胞瘤和甲状旁腺功能亢进症。此类综合征患者的麻醉管理经验不足，特别是对于接受非嗜铬细胞瘤切除术的嗜铬细胞瘤患者。我们旨在分享我们对 MEN2A 患者麻醉管理的经验。
我们回顾性地纳入了 2015 年 1 月 1 日至 2021 年 12 月 31 日期间在北京协和医院接受不同类型手术的 24 例 MEN2A 患者。所有病历均进行了回顾和分析。
共有 24 例 MEN2A 患者接受了 33 次手术，其中 20 次手术包括 17 例嗜铬细胞瘤切除术。这些接受过嗜铬细胞瘤切除术的患者在手术和麻醉过程中大多出现典型的血流动力学变化。对于其余 13 例 13 名患者的非嗜铬细胞瘤切除术，10 例在无嗜铬细胞瘤的患者中进行，3 例手术在功能原发性（1 例，双侧肿瘤，患者拒绝肾上腺切除术）或转移性嗜铬细胞瘤（2 例，双侧肾上腺切除术后多年不可切除和恶性肿瘤）中进行。对于后 3 例患者，1 例在麻醉诱导时出现高血压和心动过速，1 例在手术中出现心动过速，另 1 例在手术中表现稳定。接受嗜铬细胞瘤切除术的患者（n=17）的术后住院时间长于接受无嗜铬细胞瘤的非嗜铬细胞瘤切除术的患者（n=10）（5.8±1.8 比 4.3±1.6；P=0.031）。
无论 MEN2A 患者何时被诊断为嗜铬细胞瘤，手术切除嗜铬细胞瘤仍然是 MEN2A 治疗的首选方法。对于有现存嗜铬细胞瘤的非嗜铬细胞瘤手术可能存在风险，需要充分警惕和准备。
