Background and Objective: Primary adrenal lymphoma is a rare, progressive, easily misdiagnosed adrenal tumor with a poor prognosis. There are limited data on its clinical characteristics, and these have been derived from small sample studies. This study aimed to identify the clinical characteristics and prognosis of primary adrenal lymphoma. Methods: This single-center study retrospectively analyzed data of 50 primary adrenal lymphoma patients treated between January 2008 and January 2018. Demographic information, biochemical indexes, computed tomography images, pathological findings, treatment regimens, and prognostic factors were analyzed. Results: The median age of onset was 60.3 years, and 30 (60.0%) of 50 patients were male. Abdominal pain was the most common symptom, followed by incidentaloma and B symptoms. On average, patients presented with elevated lactate dehydrogenase (348 IU/L, normal range 110-220 IU/L) and hydroxybutyrate dehydrogenase levels (287 IU/L, normal range 72-182 IU/L) and decreased high-density lipoprotein cholesterol levels (0.88 mmol/L, normal range > 0.9 mmol/L). Bilateral lesions in the adrenal glands were observed in 30 (60.0%) patients. Computed tomography showed that 42 (84%) patients had signs of infiltration. Diffuse large B-cell lymphoma was present in 44 (88%) patients. Immunohistochemistry revealed that 70.6% (12/17), 89.5% (17/19), 92.0% (23/25), and 68.8% (11/16) of patients were positive for MYC, p53, BCL2, and both MYC and BCL2, respectively. Combined chemotherapy was associated with a good prognosis. Conclusions: Early diagnosis of primary adrenal lymphoma depends on a combination of biochemical examination, imaging studies, and pathological biopsy, and combined chemotherapy may lead to a better prognosis.
Objectives: The current study aimed to investigate the value of the computed tomography-based left-versus-right adrenal gland volume ratio (L/Rv) in screening patients with unilateral primary aldosteronism. Methods: The current study recruited 114 patients who underwent successful adrenal venous sampling (AVS) and adrenal computed tomography at West China Hospital of Sichuan University. The patients were divided into three groups according to the AVS results: AVS-left, AVS-bilateral, and AVS-right primary aldosteronism. The volumes of the left and right adrenal glands were semiautomatically calculated. The L/Rv of each patient was computed, and its value in identifying unilateral primary aldosteronism was analyzed. Results: The mean value of the L/Rv was larger in AVS-left patients and smaller in AVS-right patients than that in AVS-bilateral patients. In AVS-left primary aldosteronism patients, the cutoff value of the L/Rv with the highest Youden index was 1.344 [area under the curve (AUC) 0.851, sensitivity 80.0%, specificity 78.1%]. The optimal cutoff value was 1.908, of which 46.0% (23/50) of AVS-left primary aldosteronism patients could be identified (specificity 100.0%). In AVS-right primary aldosteronism patients, the cutoff value of the L/Rv with the highest Youden index was 1.267 (AUC 0.868, specificity 72.8%, sensitivity 87.9%). The optimal cutoff value was 0.765, of which 27.3% (9/33) of AVS-right primary aldosteronism patients could be identified (specificity 100.0%). Patients with L/Rv more than 1.908 or less than 0.765 had higher complete success rate postsurgery. Conclusion: Although not perfect, the L/Rv is an applicable index to screen unilateral primary aldosteronism patients for surgery. Primary aldosteronism patients, even those aged more than 35 years, with an L/Rv more than 1.908 or less than 0.765 can be spared AVS before surgery.
Aldosterone-to-renin ratio is the most reliable screening method of primary aldosteronism and has been widely used in clinical practice, but the index is influenced by many factors, some of which cause it false-negative, consequently leading to primary aldosteronism underdiagnosed. We report a rare case of a 27-year-old woman complaining of elevated arterial blood pressure and spontaneous hypokalemia but whose aldosterone-to-renin ratio were negative consecutively. She also had symptoms of polydipsia and polyuria for more than 20 years, with the volume of water intake and urine output up to 17 liters per day. Confirmatory tests of saline infusion test and captopril challenge test could not suppress plasma aldosterone concentration to the cutoff value. Abdominal contrast-enhanced CT suggested an adenoma on the right adrenal gland.After excluding other known causes of hypertension with hypokalemia, the patient was ultimately diagnosed with aldosterone-producing adenoma complicated with primary polydipsia. Complete clinical remission was achieved after unilateral adrenalectomy. The histopathology showed typical features of adrenocortical adenoma which was positive for CYP11B2 by immunohistochemistry, and next-generation sequencing results of tumor tissues revealed a missense mutation of the KCNJ5 gene [chr11:128781619, c.451 (exon 2) G>A]. All these findings supported the diagnosis of aldosterone-producing adenoma. This study has shown that negative aldosterone-to-renin ratio screening result cannot simply exclude primary aldosteronism.Comprehensive patient's evaluation should be taken to avoid missed diagnosis in clinical work, especially for those who have potentially curative surgery.
Context Primary adrenal lymphoma (PAL) is difficult to distinguish from other adrenal masses. Soluble interleukin-2 receptor (sIL-2R) is a diagnostic biomarker for nodal non-Hodgkin lymphoma, whose association with PAL is unknown. Objective The aim of this study was to determine the diagnostic utility of serum sIL-2R for Patients with PAL. Design Prospective cohort study. Settings and Participants A total of 118 patients with adrenal masses who were willing to be tested for levels of serum sIL-2R from a tertiary hospital between 2019 and 2021 were included. Main Outcomes and Measures Serum sIL-2R and lactate dehydrogenase (LDH) levels. Results Patients with PAL had significantly higher sIL-2R levels than those of patients with other adrenal masses with indetermined and benign CT features (both p<0.001). The LDH levels of patients with PAL were also significantly higher than those of patients with other adrenal masses with indeterminate and benign CT features (both p<0.001). Good discrimination of patients with PAL from other patients (PAL vs. other adrenal masses with indeterminate CT features/non-PAL) was achieved with an area under the receiver operating characteristic (ROC) curve (AUC) of 0.984 (95% CI, 0.95-1)/0.992 (95% CI, 0.975-1) using the serum levels of sIL-2R and further improved (AUC=0.998, 95% CI, 0.994-1; AUC=0.999, 95% CI, 0.996-1) after adjusting by LDH category. Conclusions For the first time, we have identified that serum sIL-2R and LDH category-adjusted sIL-2R levels have good diagnostic performances for PAL.
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