title: 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.
authors.affiliation: Program on Developmental Endocrinology and Genetics, Section on Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, MD, USA.
authors.forename: P P
authors.initials: PP
authors.lastname: Chrysostomou
authors.affiliation: Program on Developmental Endocrinology and Genetics, Section on Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, MD, USA.
authors.forename: M B
authors.initials: MB
authors.lastname: Lodish
authors.affiliation: Radiology and Imaging Sciences, Clinical Center, and National Institute of Biomedical Imaging and Bioengineering, National Institutes of Health, Bethesda, MD, USA.
authors.forename: E B
authors.initials: EB
authors.lastname: Turkbey
authors.affiliation: Department of Radiology and Imaging Sciences, Clinical Center, National Institutes of Health, Bethesda, MD, USA.
authors.forename: G Z
authors.initials: GZ
authors.lastname: Papadakis
authors.affiliation: Program on Developmental Endocrinology and Genetics, Section on Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, MD, USA.
authors.forename: C A
authors.initials: CA
authors.lastname: Stratakis
mesh_terms: D000293:Adolescent; D000311:Adrenal Glands / Q000033:anatomy & histology / Q000254:growth & development*; D000312:Adrenal Hyperplasia, Congenital / Q000000981:diagnostic imaging* / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology; D000328:Adult; D002648:Child; D002675:Child, Preschool; D054893:Cone-Beam Computed Tomography; D003480:Cushing Syndrome / Q000000981:diagnostic imaging* / Q000235:genetics / Q000378:metabolism / Q000503:physiopathology; D054756:Cyclic AMP-Dependent Protein Kinase RIalpha Subunit / Q000235:genetics / Q000378:metabolism; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D009929:Organ Size; D055815:Young Adult
chemical_list: D054756:Cyclic AMP-Dependent Protein Kinase RIalpha Subunit; D006854:Hydrocortisone