Abstract
ObjectivePrimary 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.Methods and ResultsAt 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.ConclusionsThe 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.
Highlights
Primary adrenal lymphoma (PAL) is a rare adrenal malignancy constituting 1% of non-Hodgkin’s lymphoma (NHL), and its most common histological type is diffuse large B cell lymphoma (DLBCL) [1, 2]
In this study, we retrospectively reviewed the medical records of two independent cohorts of patients with nonfunctional adrenal masses and functional PCC from the same institution and aimed to establish a novel diagnostic model for PAL based on clinical, laboratory and radiological data
All adrenal masses were defined as nonfunctional when 1) urine or serum free cortisol and adrenocorticotropic hormone were in the normal reference range, the cortisol circadian rhythm was normal, or cortisol was suppressed in the small-dose dexamethasone suppression test; 2) the ratio of plasma aldosterone to renin activity or the aldosterone level did not meet the primary aldosteronoma definition according to the diagnostic criteria; and 3) urine or serum epinephrine or norepinephrine was less than 3 times the upper limit of the reference range [22,23,24]
Summary
Primary adrenal lymphoma (PAL) is a rare adrenal malignancy constituting 1% of non-Hodgkin’s lymphoma (NHL), and its most common histological type is diffuse large B cell lymphoma (DLBCL) [1, 2]. PAL refers to histopathologically confirmed adrenal lymphoma with no previously diagnosed lymphoma at other sites or with coinstantaneous less-predominant lesions at sites other than the adrenal glands [3, 4]. The manifestations of PAL are not discriminating, leaving a diagnostic challenge for patients, endocrinologists and surgeons to distinguish PAL from other adrenal masses [6]. Few studies have focused on the differentiation of malignant tumors. In such cases, adrenal biopsy or fine needle aspiration (FNA) may be a diagnostic option when PCC can be fully excluded [14]. To diagnose PAL, it is essential to screen for PAL in patients with positive catecholamine results and to distinguish patients with PAL from patients with other nonfunctional adrenal masses
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