Department of Internal Medicine, Division of Endocrinology, Radboud University Medical Center, 6525 GA Nijmegen, The Netherlands.
Department of Urology, Radboud University Medical Center, 6525 GA Nijmegen, The Netherlands.
Eur J Endocrinol. 2024 Aug 30;191(3):345-353. doi: 10.1093/ejendo/lvae108.
Hereditary pheochromocytoma (hPCC) commonly develops bilaterally, causing adrenal insufficiency when standard treatment, radical adrenalectomy (RA), is performed. Partial adrenalectomy (PA) aims to preserve adrenal function, but with higher recurrence rates. This study compares outcomes of PA versus RA in hPCC.
Patients with hPCC due to pathogenic variants in RET, VHL, NF1, MAX, and TMEM127 from 12 European centers (1974-2023) were studied retrospectively. Stratified analysis based on surgery type and initial presentation was conducted. The main outcomes included recurrence, adrenal insufficiency, metastasis, and mortality.
The study included 256 patients (223 RA, 33 PA). Ipsilateral recurrence rates were 9/223 (4%) after RA versus 5/33 (15%) after PA (P = 0.02). Metastasis and mortality did not differ between groups. Overall, 103 patients (40%) underwent bilateral adrenalectomy either synchronously or metachronously (75 RA, 28 PA). Of these, 46% developed adrenal insufficiency after PA.In total, 191 patients presented with initial unilateral disease, of whom 50 (26%) developed metachronous contralateral disease, most commonly in RET, VHL, and MAX. In patients with metachronous bilateral disease, adrenal insufficiency developed in 3/4 (75%) when PA was performed as the first operation followed by RA, compared to 1/7 (14%) when PA was performed as the second operation after prior RA (P = 0.09).
In patients with hPCC undergoing PA, local recurrence rates are higher than after RA, but metastasis and disease-specific mortality are similar. Therefore, PA seems a safe method to preserve adrenal function in patients with hPCC, in cases of both synchronous and metachronous bilateral disease, when performed as a second operation.
遗传性嗜铬细胞瘤（hPCC）常双侧发生，当采用标准治疗方法——根治性肾上腺切除术（RA）时，会导致肾上腺功能不全。部分肾上腺切除术（PA）旨在保留肾上腺功能，但复发率较高。本研究比较了 hPCC 中 PA 与 RA 的治疗结果。
对来自 12 个欧洲中心（1974 年至 2023 年）的因 RET、VHL、NF1、MAX 和 TMEM127 种系变异导致 hPCC 的患者进行回顾性研究。根据手术类型和初始表现进行分层分析。主要结局包括复发、肾上腺功能不全、转移和死亡。
本研究纳入 256 例患者（223 例行 RA，33 例行 PA）。RA 后同侧复发率为 9/223（4%），PA 后为 5/33（15%）（P = 0.02）。两组间转移和死亡率无差异。总体而言，103 例患者（40%）行双侧肾上腺切除术，包括同期或异期手术（75 例行 RA，28 例行 PA）。其中，46%的患者在 PA 后发生肾上腺功能不全。共有 191 例患者初诊为单侧疾病，其中 50 例（26%）发生对侧疾病，最常见于 RET、VHL 和 MAX。在发生对侧双侧疾病的患者中，当 PA 作为第一手术，随后行 RA 时，3/4（75%）患者发生肾上腺功能不全，而当 PA 作为 RA 后第二手术时，1/7（14%）患者发生肾上腺功能不全（P = 0.09）。
在接受 PA 的 hPCC 患者中，局部复发率高于 RA，但转移和疾病特异性死亡率相似。因此，PA 似乎是一种安全的方法，可在双侧疾病同时或异时发生时，作为第二手术保留 hPCC 患者的肾上腺功能。
