双侧嗜铬细胞瘤很少见，且通常具有遗传性。全肾上腺切除术可实现彻底的肿瘤治愈，并导致随后的明确的皮质醇功能减退症。肾上腺次全切除术是一种可能的选择。本研究的目的是评估肾上腺全切除术和肾上腺次全切除术对双侧嗜铬细胞瘤术后复发率、转移性疾病和类固醇依赖性的影响。在文献数据库 PubMed、Embase 和 Europe PMC 中系统检索1945 年至 2023 年 6 月 1 日进行。遵循 PRISMA 指南，并将 PICO 策略应用于比较肾上腺次全切除术和肾上腺全切除术的英语研究。使用随机效应模型来评估具有高异质性的研究的不同结果。使用纽卡斯尔-渥太华量表和非随机干预研究中的偏倚风险 (“ROBINS-I”) 工具来评估质量和偏倚风险。在总共 12 909 项研究中，1202 名患者（来自 10 项回顾性研究） ）有资格进行荟萃分析。在包括 1176 名患者的六项研究中，肾上腺次全切除术和肾上腺全切除术后的复发率分别为 14.1% 和 2.6%（OR 4.91，95% c.i. 1.30 至 18.54；P = 0.020；I2 72%）。在包括 1124 名患者的 9 项研究中，肾上腺全切除术和肾上腺次全切除术后的术后类固醇依赖率分别为 93.3% 和 11.6%（OR 0.003，95% c.i. 0.0003 至 0.03；P < 0.00001；I2 66%） 。根据包括 719 名患者的两项研究，术后转移性疾病的发生率没有明显差异。肾上腺次全切除术可减少术后原发性肾上腺皮质功能不全，但术后复发率较高。未来需要具有明确资格标准的前瞻性随机研究来证实这些结果。© 作者 2023。由牛津大学出版社代表 BJS Society Ltd 出版。

Bilateral pheochromocytomas are rare and often heritable. Total adrenalectomy leads to a definitive oncological cure, with subsequent definitive hypocortisolism. Subtotal adrenalectomy is a possible alternative. The aim of this study was to assess the effects of total adrenalectomy and subtotal adrenalectomy on bilateral pheochromocytoma in terms of post-surgical rate of recurrence, metastatic disease, and steroid dependence.Systematic searches in the bibliographic databases PubMed, Embase, and Europe PMC were performed for 1945 to 1 June 2023. PRISMA guidelines were followed and the PICO strategy was applied to English-language studies comparing subtotal adrenalectomy with total adrenalectomy. A random-effects model was used to assess the different outcomes for studies with high heterogeneity. The Newcastle-Ottawa scale and the Risk Of Bias In Non-randomized Studies of Interventions ('ROBINS-I') tool were used to assess quality and risk of bias.From a total of 12 909 studies, 1202 patients (from 10 retrospective studies) were eligible for the meta-analysis. In six studies, including 1176 patients, the recurrence rate after subtotal adrenalectomy and total adrenalectomy was 14.1 versus 2.6 per cent respectively (OR 4.91, 95 per cent c.i. 1.30 to 18.54; P = 0.020; I2 72 per cent). In nine studies, including 1124 patients, the rate of post-surgical steroid dependence was 93.3 versus 11.6 per cent after total adrenalectomy and subtotal adrenalectomy respectively (OR 0.003, 95 per cent c.i. 0.0003 to 0.03; P < 0.00001; I2 66 per cent). Based on two studies, including 719 patients, no differences were evident regarding the occurrence of post-surgery metastatic disease.Subtotal adrenalectomy leads to less post-surgical primary adrenal insufficiency, but leads to a higher postoperative recurrence rate. Future prospective randomized studies, with clear eligibility criteria, are needed to confirm these results.© The Author(s) 2023. Published by Oxford University Press on behalf of BJS Society Ltd.