Adrenal Center, Department of Endocrinology and Metabolism, West China Hospital, Sichuan University, Chengdu, China.
Department of Public Health, Chengdu Medical College, Chengdu, China.
Front Endocrinol (Lausanne). 2021 Apr 2;12:636658. doi: 10.3389/fendo.2021.636658. eCollection 2021.
Primary adrenal lymphoma (PAL) is easily misdiagnosed as other adrenal masses, such as adrenocortical carcinoma and pheochromocytoma, but patients with PAL benefit little from surgery. The diagnostic method for PAL thus far is limited to adrenal biopsy. In our study, we aimed to develop a quick and efficient diagnostic method for PAL.
At the same institution, 505 patients (between 2009 and 2019) and 171 patients (between 2019 and 2020) were separately included in the primary and validation studies. Univariate and multivariate analyses were conducted to evaluate clinical manifestations, laboratory findings, and radiological characteristics. Four determinants (age, bilateral masses, high-density lipoprotein cholesterol, and lactate dehydrogenase) were selected and further incorporated into a regression model to screen PAL. Accordingly, the nomogram was developed for clinical practice. In the primary study, the nomogram showed good discrimination, with an area under the receiver operating characteristic (ROC) curve (AUC) of 95.4% (95% CI, 90.6%-100.0%). Further validation study verified the efficacy of the nomogram, with an AUC of 99.0% (95% CI, 96.9%-100.00%) and 100.0% in all patients and patients with bilateral masses, respectively, and a sensitivity/specificity/positive predictive value (PPV)/negative predictive value (NPV) of 66.67%/99.40%/66.67%/99.40%, 66.67%/100%/100%/92.86%, 50%/99.20%/50%/99.20%, and 100%/100%/100%/100%, in all patients, patients with bilateral adrenal masses, patients with nonfunctional adrenal masses, and patients with positive catecholamine results, respectively. The validation study also revealed a diagnostic specificity of 99.35% and 100% for patients with a unilateral adrenal mass and functional PCC, respectively.
The presented nomogram is the first user-friendly diagnostic model for PAL that simplifies the complex diagnostic process into personalized numeric estimates. We deem that patients who score below 50 are less likely to have PAL. We suggest that clinicians should arrange adrenal biopsy and surgery for patients with nonfunctional tumors and overt catecholamine-secreting tumors, respectively, who receive a score of 50 points or higher to confirm the diagnosis as soon as possible.
原发性肾上腺淋巴瘤（PAL）很容易被误诊为其他肾上腺肿块，如肾上腺皮质癌和嗜铬细胞瘤，但 PAL 患者从手术中获益甚微。迄今为止，PAL 的诊断方法仅限于肾上腺活检。在本研究中，我们旨在开发一种快速有效的 PAL 诊断方法。
在同一机构，分别纳入了 505 例（2009 年至 2019 年）和 171 例（2019 年至 2020 年）患者进行主要和验证研究。进行了单变量和多变量分析，以评估临床表现、实验室检查和影像学特征。选择了 4 个决定因素（年龄、双侧肿块、高密度脂蛋白胆固醇和乳酸脱氢酶），并进一步纳入回归模型以筛选 PAL。据此，为临床实践制定了列线图。在主要研究中，该列线图显示出良好的区分度，受试者工作特征（ROC）曲线下面积（AUC）为 95.4%（95%CI，90.6%-100.0%）。进一步的验证研究验证了该列线图的有效性，在所有患者和双侧肾上腺肿块患者中，AUC 分别为 99.0%（95%CI，96.9%-100.00%）和 100.0%，在所有患者和双侧肾上腺肿块患者中的敏感性/特异性/阳性预测值（PPV）/阴性预测值（NPV）分别为 66.67%/99.40%/66.67%/99.40%、66.67%/100%/100%/92.86%、50%/99.20%/50%/99.20%和 100%/100%/100%/100%，在所有患者、双侧肾上腺肿块患者、无功能性肾上腺肿块患者和儿茶酚胺阳性患者中分别为 50%/99.20%/50%/99.20%。验证研究还显示，单侧肾上腺肿块和功能性 PCC 患者的诊断特异性分别为 99.35%和 100%。
本研究提出的列线图是 PAL 的第一个易于使用的诊断模型，它将复杂的诊断过程简化为个性化的数值估计。我们认为评分低于 50 分的患者不太可能患有 PAL。我们建议对于评分在 50 分或以上的无功能性肿瘤和明显儿茶酚胺分泌性肿瘤患者，临床医生应安排进行肾上腺活检和手术，以尽快明确诊断。
