CONTEXT: Patients with 21-hydroxylase deficiency (21-OHD) have been shown to develop adrenal adenomas and, in males, testicular adrenal rest tumors (TARTs) at a high percentage. OBJECTIVE: The aim of this study was to evaluate the interrelation of adrenal masses and TARTs as well as factors stimulating tumor growth of orthotopic and ectopic adrenal tissue in 21-OHD. DESIGN: In a cross-sectional study, 26 adult male patients with classic 21-OHD (15 salt wasting, 11 simple virilizing; age range, 18-48 yr) were clinically assessed according to their hormonal control. Magnetic resonance imaging of the adrenals (26 of 26) and of the testes (18 of 26) was performed. Adrenal size and morphology was compared to 26 age-matched controls. RESULTS: Combined adrenal volume of 21-OHD patients was significantly higher (median, 9.3 ml; range, 3.2-124.5 ml) in comparison to controls (median, 7.4 ml; range, 5.5-10.8 ml; P = 0.005). Morphologically, adrenals were classified as normal without nodules in 27% of 21-OHD patients compared to 69% of controls. None of the controls, but 42% of 21-OHD patients had an overall adrenal volume higher than 11 ml. Ten of 18 patients had TARTs with a median volume of 3.3 ml (range, 0.4-21.6 ml). Total adrenal volume and tumor size but not TART volume correlated positively with current parameters of hormonal control (androstenedione, morning 17-OHP in serum, pregnanetriol in 24-h urine; P < 0.001 for each). Baseline ACTH was independent of adrenal and TART volume. There was no correlation of total adrenal or adrenal tumor size with TART volume. CONCLUSION: These data provide indirect evidence that different factors regulate the growth of orthotopic adrenal tissue and ectopic adrenal remnants in TARTs.
CONTEXT: Patients with 21-hydroxylase deficiency (21-OHD) have been shown to develop adrenal adenomas and, in males, testicular adrenal rest tumors (TARTs) at a high percentage. OBJECTIVE: The aim of this study was to evaluate the interrelation of adrenal masses and TARTs as well as factors stimulating tumor growth of orthotopic and ectopic adrenal tissue in 21-OHD. DESIGN: In a cross-sectional study, 26 adult male patients with classic 21-OHD (15 salt wasting, 11 simple virilizing; age range, 18-48 yr) were clinically assessed according to their hormonal control. Magnetic resonance imaging of the adrenals (26 of 26) and of the testes (18 of 26) was performed. Adrenal size and morphology was compared to 26 age-matched controls. RESULTS: Combined adrenal volume of 21-OHDpatients was significantly higher (median, 9.3 ml; range, 3.2-124.5 ml) in comparison to controls (median, 7.4 ml; range, 5.5-10.8 ml; P = 0.005). Morphologically, adrenals were classified as normal without nodules in 27% of 21-OHDpatients compared to 69% of controls. None of the controls, but 42% of 21-OHDpatients had an overall adrenal volume higher than 11 ml. Ten of 18 patients had TARTs with a median volume of 3.3 ml (range, 0.4-21.6 ml). Total adrenal volume and tumor size but not TART volume correlated positively with current parameters of hormonal control (androstenedione, morning 17-OHP in serum, pregnanetriol in 24-h urine; P < 0.001 for each). Baseline ACTH was independent of adrenal and TART volume. There was no correlation of total adrenal or adrenal tumor size with TART volume. CONCLUSION: These data provide indirect evidence that different factors regulate the growth of orthotopic adrenal tissue and ectopic adrenal remnants in TARTs.
Authors: Richard J Auchus; Elizabeth O Buschur; Alice Y Chang; Gary D Hammer; Carole Ramm; David Madrigal; George Wang; Martha Gonzalez; Xu Steven Xu; Johan W Smit; James Jiao; Margaret K Yu Journal: J Clin Endocrinol Metab Date: 2014-04-29 Impact factor: 5.958
Authors: Melissa K Crocker; Stephanie Barak; Corina M Millo; Stephanie A Beall; Mahtab Niyyati; Richard Chang; Nilo A Avila; Carol Van Ryzin; James Segars; Martha Quezado; Deborah P Merke Journal: J Clin Endocrinol Metab Date: 2012-08-17 Impact factor: 5.958