Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA.
Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN, USA.
Clin Endocrinol (Oxf). 2021 Jul;95(1):47-57. doi: 10.1111/cen.14460. Epub 2021 Mar 22.
To describe the presentation and outcomes of patients with adrenal ganglioneuromas (AGNs).
Single-centre retrospective cohort study (1 January 1995 to 31 December 2019) and systematic review of literature (1 January 1980 to 19 November 2019).
Diagnosed with histologically confirmed AGN.
Baseline clinical, imaging and biochemical characteristics, recurrence rates and mortality. Subgroup analysis was performed on tumours with histologic elements of ganglioneuroma and pheochromocytoma (ie composite tumours).
The cohort study included 45 patients with AGN, 20 (44%) of which had composite tumours. Compared to pure AGN, patients with composite tumour were older (median age, 62.5 vs. 35 years, p < .001), had smaller tumours (median size, 3.9 vs. 5.7 cm, p = .016) and were discovered incidentally less frequently (65% vs. 84%, p = .009). No recurrences or ganglioneuroma-specific mortality occurred during follow-up (range, 0-266 months). The systematic review included 14 additional studies and 421 patients. The mean age of diagnosis was 39 years, and 47% were women. AGNs were discovered incidentally in 72% of patients, were predominantly unilateral (99%) and had a mean diameter of 5.8 cm and an unenhanced computed tomography (CT) attenuation of -118 to 49 Hounsfield units (HU). On imaging, 69% of AGNs were homogenous, 41% demonstrated calcifications, and 40% were lobulated.
AGNs are rare benign tumours that present with variable imaging features including large size, unenhanced CT attenuation >20 HU, calcifications and lobulated shape. Imaging characteristics can assist in establishing a diagnosis and avoiding an unnecessary adrenalectomy. The association of pheochromocytomas with AGNs is frequent. Diagnosis should include biochemical testing.
描述肾上腺节细胞神经瘤（AGN）患者的表现和结局。
单中心回顾性队列研究（1995 年 1 月 1 日至 2019 年 12 月 31 日）和文献系统回顾（1980 年 1 月 1 日至 2019 年 11 月 19 日）。
经组织学证实为 AGN。
基线临床、影像学和生化特征、复发率和死亡率。对具有节细胞神经瘤和嗜铬细胞瘤组织学特征（即复合肿瘤）的肿瘤进行了亚组分析。
该队列研究纳入了 45 例 AGN 患者，其中 20 例（44%）为复合肿瘤。与单纯 AGN 相比，复合肿瘤患者年龄更大（中位年龄 62.5 岁 vs. 35 岁，p<0.001），肿瘤更小（中位大小 3.9 厘米 vs. 5.7 厘米，p=0.016），且偶然发现的频率更低（65% vs. 84%，p=0.009）。在随访期间（0-266 个月）未发生复发或与节细胞神经瘤相关的死亡率。文献系统综述纳入了另外 14 项研究和 421 例患者。诊断时的平均年龄为 39 岁，女性占 47%。72%的患者为偶然发现，肿瘤均为单侧（99%），平均直径为 5.8 厘米，未增强 CT 衰减值为-118 至 49 亨氏单位（HU）。影像学上，69%的 AGN 呈均匀性，41%有钙化，40%呈分叶状。
AGN 是罕见的良性肿瘤，表现为多种影像学特征，包括大肿瘤、未增强 CT 衰减值>20 HU、钙化和分叶状。影像学特征有助于建立诊断，避免不必要的肾上腺切除术。AGN 常与嗜铬细胞瘤相关。诊断应包括生化检查。
