Use of 3-Dimensional Volumetric Modeling of Adrenal Gland Size in Patients with Primary Pigmented Nodular Adrenocortical Disease

Abstract

Primary pigmented nodular adrenocortical disease (PPNAD) is a rare type of bilateral adrenal hyperplasia leading to hypercortisolemia. Adrenal nodularity is often appreciable with computed tomography (CT); however, accurate radiologic characterization of adrenal size in PPNAD has not been studied well. We used 3-dimensional (3D) volumetric analysis to characterize and compare adrenal size in PPNAD patients, with and without Cushing's syndrome (CS). Patients diagnosed with PPNAD and their family members with known mutations in PRKAR1A were screened. CT scans were used to create 3D models of each adrenal. Criteria for biochemical diagnosis of CS included loss of diurnal variation and/or elevated midnight cortisol levels, and paradoxical increase in urinary free cortisol and/or urinary 17-hydroxysteroids after dexamethasone administration. Forty-five patients with PPNAD (24 females, 27.8±17.6 years) and 8 controls (19±3 years) were evaluated. 3D volumetric modeling of adrenal glands was performed in all. Thirty-eight patients out of 45 (84.4%) had CS. Their mean adrenal volume was 8.1 cc±4.1, 7.2 cc±4.5 (p=0.643) for non-CS, and 8.0cc±1.6 for controls. Mean values were corrected for body surface area; 4.7 cc/kg/m(2)±2.2 for CS, and 3.9 cc/kg/m(2)±1.3 for non-CS (p=0.189). Adrenal volume and midnight cortisol in both groups was positively correlated, r=0.35, p=0.03. We conclude that adrenal volume measured by 3D CT in patients with PPNAD and CS was similar to those without CS, confirming empirical CT imaging-based observations. However, the association between adrenal volume and midnight cortisol levels may be used as a marker of who among patients with PPNAD may develop CS, something that routine CT cannot do.

11-Oxygenated Androgens Are Biomarkers of Adrenal Volume and Testicular Adrenal Rest Tumors in 21-Hydroxylase Deficiency

“…This tracing included any bulges of the contour caused by nodules or masses in the adrenal periphery. The adrenal volume was calculated from the sum of each axial slice area ( 24 ). Adrenal hypertrophy was defined as volumes greater than published norms: right adrenal gland, 5.7 mL for males and 4.9 mL for females; left adrenal gland, 5.7 mL for males, 4.4 mL for females ( 25 ).…”

Adrenal morphology and associated comorbidities in congenital adrenal hyperplasia

“…This tracing included any bulges of the contour caused by nodules or masses in the adrenal periphery. The adrenal volume was calculated from the sum of each axial slice area …”

“…This tracing included any bulges of the contour caused by nodules or masses in the adrenal periphery. The adrenal volume was calculated from the sum of each axial slice area 19,20. Adrenal morphology was categorized as normal, atrophy, hyperplasia with or without nodules (<5mm), adrenocortical adenomas (≥5mm), myelolipoma or other.…”

Parathyroid Hormone Resistance and Bilateral Macronodular Adrenocortical Disease: Does Partial Loss of Methylation at the GNAS Exon 1 Differentially Methylated Region (DMR) Play a Role?

“…Recently, an association between 25-OH vitamin D levels and the secretion of cortisol and aldosterone from the adrenal gland has been reported [22]. Adrenal gland size can be assessed by three-dimensional volumetric modeling [23]. Interestingly, maternal Gsα mutations in mice and in patients with Albright hereditary osteodys- trophy are associated with obesity, insulin resistance, and hyperlipidemia [24].…”