Department of Endocrinology, Bnai Zion Medical Center, Haifa, Istael.
Department of Surgery, Bnai Zion Medical Center, Haifa, Israel.
Exp Clin Endocrinol Diabetes. 2020 Dec;128(12):827-834. doi: 10.1055/a-0998-7884. Epub 2019 Oct 21.
Primary bilateral macronodular adrenal hyperplasia (PBMAH) is characterized by benign bilateral enlarged adrenal masses, causing Cushing's syndrome (CS). The aim of the current article is to define the role of unilateral adrenalectomy (UA) in treating patients with CS related to PBMAH.
A PubMed database search was conducted to identify articles reporting UA to treat PBMAH. We also report cases of PBMAH from our medical center treated by UA.
A total number of 71 cases of PBMAH (62 cases reported in the literature and 9 cases from our center) are presented. Most patients were women (73.2%) and most UA involved the left side (64.3%). In most cases, the resected gland was the larger one. Following UA, 94.4% of cases had remission of hypercortisolism. Recurrence rate of CS was 19.4% and hypoadrenalism occurred in 29.6%. After UA, when the size of the remained adrenal gland was equal or greater than 3.5 cm, CS persisted in 21.4% of cases, and recurrence occurred in 27.3% of cases (after 20±9.2 months). However, when the size of the remained gland was less than 3.5 cm, CS resolved in all cases and recurrence occurred in 21.2% of cases after a long period (65.6±52.1 months). High levels of urinary free cortisol (UFC) were not correlated with post-surgical CS recurrence or persistence.
UA leads to beneficial outcomes in patients with CS related to PBMAH, also in cases with pre-surgical elevated UFC or contralateral large gland.
双侧原发性大结节性肾上腺增生（PBMAH）的特征是良性双侧肾上腺增大，导致库欣综合征（CS）。本文的目的是定义单侧肾上腺切除术（UA）在治疗与 PBMAH 相关的 CS 患者中的作用。
对 PubMed 数据库进行了检索，以确定报告 UA 治疗 PBMAH 的文章。我们还报告了我们医疗中心治疗 PBMAH 的病例。
共报告了 71 例 PBMAH 病例（文献报道 62 例，我院中心 9 例）。大多数患者为女性（73.2%），大多数 UA 涉及左侧（64.3%）。在大多数情况下，切除的腺体是较大的一个。UA 后，94.4%的病例皮质醇增多症缓解。CS 的复发率为 19.4%，肾上腺功能减退发生率为 29.6%。UA 后，当残留肾上腺的大小等于或大于 3.5cm 时，CS 持续存在于 21.4%的病例中，27.3%的病例在 20±9.2 个月后复发。然而，当残留腺体的大小小于 3.5cm 时，CS 在所有病例中均得到缓解，21.2%的病例在较长时间（65.6±52.1 个月）后复发。尿游离皮质醇（UFC）水平升高与术后 CS 复发或持续无关。
UA 对与 PBMAH 相关的 CS 患者，甚至对术前 UFC 升高或对侧大腺体的患者，均有良好的疗效。
