Volumetric Modeling of Adrenal Gland Size in Primary Bilateral Macronodular Adrenocortical Hyperplasia

Abstract

Context Radiological characterization of adrenal size in primary bilateral macronodular adrenocortical hyperplasia (PBMAH) has not been previously investigated. Objective We hypothesized that volumetric modeling of adrenal gland size may correlate with biochemical disease severity in patients with PBMAH. Secondary analysis of patients with concurrent primary aldosteronism (PA) was performed. Design A retrospective cross-sectional analysis of 44 patients with PBMAH was conducted from 2000 to 2019. Setting Tertiary care clinical research center. Patients Patients were diagnosed with PBMAH based upon clinical, genetic, and biochemical characteristics. Intervention Clinical, biochemical, and genetic data were obtained. Computed tomography scans were used to create volumetric models by manually contouring both adrenal glands in each slice using Vitrea Core Fx v6.3 software (Vital Images, Minnetonka, Minnesota). Main Outcome and Measures. 17-hydroxycorticosteroids (17-OHS), ARMC5 genetics, and aldosterone-to-renin ratio (ARR) were retrospectively obtained. Pearson test was used for correlation analysis of biochemical data with adrenal volume. Results A cohort of 44 patients with PBMAH was evaluated, with a mean age (±SD) of 53 ±11.53. Eight patients met the diagnostic criteria for PA, of which 6 (75%) were Black. In the Black cohort, total adrenal volumes positively correlated with midnight cortisol (R=0.76, p=0.028), UFC (R=0.70, p=0.035) and 17-OHS (R=0.87, p=0.0045), with a more pronounced correlation with left adrenal volume alone. 17-OHS concentration positively correlated with total, left, and right adrenal volume in patients harboring pathogenic variants in ARMC5 (R=0.72, p=0.018; R=0.65, p=0.042; and R=0.73, p=0.016, respectively). Conclusions Volumetric modeling of adrenal gland size may associate with biochemical severity in patients with PBMAH, with particular utility in Black patients.

Steroid profiling using liquid chromatography mass spectrometry during adrenal vein sampling in patients with primary bilateral macronodular adrenocortical hyperplasia

“…This is in line with the findings of Wurth et al. in a cohort of 44 patients with PBMAH, in which adrenal volume was calculated based on computed tomography scans ( 24 ). 17-hydroxyprogesterone, DHEA, DHEAS, androstenedione and testosterone showed a positive correlation with UFC.…”

Adrenal venous sampling for lateralization of cortisol hypersecretion in patients with bilateral adrenal masses

“…AVS was found to be a safe method for cortisol lateralization, and laterality could not be assessed solely on CT findings. A study of 44 described the use of adrenal volume correlating with biochemical severity in patients with PBMAH 27 . In our study, there was a significant correlation between the right adrenal dominant nodule maximum diameter and cortisol levels, but no significant correlation between cortisol levels and adrenal gland size or left dominant nodule size.…”

“…on CT findings. A study of 44 described the use of adrenal volume correlating with biochemical severity in patients with PBMAH 27. In our study, there was a significant correlation between the right adrenal dominant nodule maximum diameter and cortisol levels, but no significant correlation between cortisol levels and adrenal gland size or left dominant nodule size.T A B L E 5Abbreviations: ACTH, corticotropin; AVS, adrenal vein sampling; N/A, not available.…”

Pathophysiology and histopathology of primary aldosteronism

“…Germline pathogenic variants in ARMC5 (encoding the armadillo repeat-containing protein 5) are frequent in cortisol-producing primary bilateral macronodular adrenal hyperplasia [56][57][58][59][60]. Of note is the presence of ARMC5 germline variants in bilateral adrenocortical nodules in African American patients with PA, but not in Caucasians [61,62].…”