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Adrenocortical hyperplasia: a review of clinical presentation and imaging

  • Special Section: Adrenal Gland
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Abstract

Adrenal hyperplasia is non-malignant enlargement of the adrenal glands, which is often bilateral. It can be incidental or related to indolent disease process and may be related to benign or malignant etiologies causing biochemical alterations in the hypothalamic–pituitary–adrenal axis which controls steroidogenesis and in particular cortisol production. Clinical significance of the adrenal hyperplasia is variable ranging from asymptomatic finding to serious manifestations of Cushing syndrome. This is often associated with anatomical changes in the adrenal glands, which typically manifests as diffuse and sometimes nodular enlargement of the adrenal glands radiologically. Approaching adrenal hyperplasia requires careful clinical and biochemical evaluation in correlation with imaging review to differentiate ACTH-dependent and ACTH-independent etiologies. CT is the primary modality of choice for adult adrenal imaging owing to reproducibility, temporal and spatial resolution and broader access, while MRI often serves a complimentary role. Ultrasound and MRI are most commonly used in pediatric cases to evaluate congenital adrenal hyperplasia. This article will discuss the clinical presentation and imaging features of different types and mimics of adrenal cortical hyperplasia.

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References

  1. Goldman SM, et al., Computed Body Tomography with MRI Correlation. Vol. 1. 2006. Philadelphia, WA.

  2. Breslow, M.J., Regulation of adrenal medullary and cortical blood flow. Am J Physiol, 1992. 262(5 Pt 2): p. H1317-30.

    CAS  PubMed  Google Scholar 

  3. Sargar, K.M., G. Khanna, and R. Hulett Bowling, Imaging of Nonmalignant Adrenal Lesions in Children. Radiographics, 2017. 37(6): p. 1648-1664.

  4. Vincent, J.M., et al., The size of normal adrenal glands on computed tomography. Clin Radiol, 1994. 49(7): p. 453-5.

    Article  CAS  Google Scholar 

  5. Li, L.L., et al., Incidental adrenal enlargement: an overview from a retrospective study in a chinese population. Int J Endocrinol, 2015. 2015: p. 192874.

    PubMed  PubMed Central  Google Scholar 

  6. Zeiger, M.A., et al., American Association of Clinical Endocrinologists and American Association of Endocrine Surgeons Medical Guidelines for the Management of Adrenal Incidentalomas: executive summary of recommendations. Endocr Pract, 2009. 15(5): p. 450-3.

    Article  Google Scholar 

  7. Kangarloo, H., et al., Sonography of adrenal glands in neonates and children: changes in appearance with age. J Clin Ultrasound, 1986. 14(1): p. 43-7.

    Article  CAS  Google Scholar 

  8. Tang, Y.Z., et al., The prevalence of incidentally detected adrenal enlargement on CT. Clin Radiol, 2014. 69(1): p. e37-42.

    Article  CAS  Google Scholar 

  9. Schteingart, D.E., The clinical spectrum of adrenocortical hyperplasia. Curr Opin Endocrinol Diabetes Obes, 2012. 19(3): p. 176-82.

    Article  CAS  Google Scholar 

  10. Michelle, M.A., et al., Adrenal cortical hyperplasia: diagnostic workup, subtypes, imaging features and mimics. Br J Radiol, 2017. 90(1079): p. 20170330.

    Article  Google Scholar 

  11. Teixeira, S.R., et al., The role of imaging in congenital adrenal hyperplasia. Arq Bras Endocrinol Metabol, 2014. 58(7): p. 701-8.

    Article  Google Scholar 

  12. Arnaldi, G., et al., Diagnosis and complications of Cushing’s syndrome: a consensus statement. J Clin Endocrinol Metab, 2003. 88(12): p. 5593-602.

    Article  CAS  Google Scholar 

  13. Nieman, L.K., et al., The diagnosis of Cushing’s syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab, 2008. 93(5): p. 1526-40.

    Article  CAS  Google Scholar 

  14. Bansal, V., et al., Pitfalls in the diagnosis and management of Cushing’s syndrome. Neurosurg Focus, 2015. 38(2): p. E4.

    Article  Google Scholar 

  15. Newell-Price, J., et al., Optimal response criteria for the human CRH test in the differential diagnosis of ACTH-dependent Cushing’s syndrome. J Clin Endocrinol Metab, 2002. 87(4): p. 1640-5.

    CAS  PubMed  Google Scholar 

  16. Wagner-Bartak, N.A., et al., Cushing Syndrome: Diagnostic Workup and Imaging Features, With Clinical and Pathologic Correlation. AJR Am J Roentgenol, 2017. 209(1): p. 19-32.

    Article  Google Scholar 

  17. Speiser, P.W., et al., Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab, 2010. 95(9): p. 4133-60.

    Article  CAS  Google Scholar 

  18. Kaye, C.I., et al., Newborn screening fact sheets. Pediatrics, 2006. 118(3): p. e934-63.

    Article  Google Scholar 

  19. Yeh, H.C., US and CT evaluation of diffusely enlarged adrenal gland. Crit Rev Diagn Imaging, 1992. 33(5): p. 437-60.

    CAS  PubMed  Google Scholar 

  20. Elsayes K, C.E., Adrenal imaging: a practical guide to diagnostic workup and spectrum of imaging findings. Appl Radiol, 2011. 40: p. 14-19.

  21. Sahdev, A., et al., Imaging in Cushing’s syndrome. Arq Bras Endocrinol Metabol, 2007. 51(8): p. 1319-28.

    Article  Google Scholar 

  22. Elsayes, K.M., et al., Practical Approach to Adrenal Imaging. Radiol Clin North Am, 2017. 55(2): p. 279-301.

    Article  Google Scholar 

  23. Kairys, N. and A. Schwell, Cushing Disease, in StatPearls. 2019: Treasure Island (FL).

  24. Chaudhary, V. and S. Bano, Imaging of the pituitary: Recent advances. Indian J Endocrinol Metab, 2011. 15 Suppl 3: p. S216-23.

    Article  Google Scholar 

  25. Chung, E.M., et al., From the radiologic pathology archives: precocious puberty: radiologic-pathologic correlation. Radiographics, 2012. 32(7): p. 2071-99.

    Article  Google Scholar 

  26. Merke, D.P. and S.R. Bornstein, Congenital adrenal hyperplasia. Lancet, 2005. 365(9477): p. 2125-36.

    Article  Google Scholar 

  27. White, P.C. and P.W. Speiser, Congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Endocr Rev, 2000. 21(3): p. 245-91.

    CAS  PubMed  Google Scholar 

  28. Stikkelbroeck, N.M., et al., High prevalence of testicular adrenal rest tumors, impaired spermatogenesis, and Leydig cell failure in adolescent and adult males with congenital adrenal hyperplasia. J Clin Endocrinol Metab, 2001. 86(12): p. 5721-8.

    Article  CAS  Google Scholar 

  29. Al-Alwan, I., et al., Clinical utility of adrenal ultrasonography in the diagnosis of congenital adrenal hyperplasia. J Pediatr, 1999. 135(1): p. 71-5.

    Article  CAS  Google Scholar 

  30. Avila, N.A., et al., Testicular adrenal rest tissue in congenital adrenal hyperplasia: findings at Gray-scale and color Doppler US. Radiology, 1996. 198(1): p. 99-104.

    Article  CAS  Google Scholar 

  31. Therrell, B.L., Jr., et al., Results of screening 1.9 million Texas newborns for 21-hydroxylase-deficient congenital adrenal hyperplasia. Pediatrics, 1998. 101(4 Pt 1): p. 583-90.

  32. Miller, W.L., Disorders in the initial steps of steroid hormone synthesis. J Steroid Biochem Mol Biol, 2017. 165(Pt A): p. 18-37.

    Article  CAS  Google Scholar 

  33. Ayala-Ramirez, M., et al., Adrenocortical carcinoma: clinical outcomes and prognosis of 330 patients at a tertiary care center. Eur J Endocrinol, 2013. 169(6): p. 891-899.

    Article  CAS  Google Scholar 

  34. Newell-Price, J., et al., Cushing’s syndrome. Lancet, 2006. 367(9522): p. 1605-17.

    Article  CAS  Google Scholar 

  35. Courcoutsakis, N., P. Prassopoulos, and C.A. Stratakis, CT findings of primary pigmented nodular adrenocortical disease: rare cause of ACTH-independent Cushing syndrome. AJR Am J Roentgenol, 2010. 194(6): p. W541.

    Article  Google Scholar 

  36. Stratakis, C.A., Adrenocortical tumors, primary pigmented adrenocortical disease (PPNAD)/Carney complex, and other bilateral hyperplasias: the NIH studies. Horm Metab Res, 2007. 39(6): p. 467-73.

    Article  CAS  Google Scholar 

  37. Rockall, A.G., et al., CT and MR imaging of the adrenal glands in ACTH-independent cushing syndrome. Radiographics, 2004. 24(2): p. 435-52.

    Article  Google Scholar 

  38. Watson, T.D., S.J. Patel, and P.M. Nardi, Case 121: familial adrenocorticotropin-independent macronodular adrenal hyperplasia causing Cushing syndrome. Radiology, 2007. 244(3): p. 923-6.

    Article  Google Scholar 

  39. Fortman, B.J., et al., Neurofibromatosis type 1: a diagnostic mimicker at CT. Radiographics, 2001. 21(3): p. 601-12.

    Article  CAS  Google Scholar 

  40. Gruber, L.M., et al., Pheochromocytoma and paraganglioma in patients with neurofibromatosis type 1. Clin Endocrinol (Oxf), 2017. 86(1): p. 141-149.

    Article  CAS  Google Scholar 

  41. Kobus, K., et al., Double NF1 inactivation affects adrenocortical function in NF1Prx1 mice and a human patient. PLoS One, 2015. 10(3): p. e0119030.

    Article  Google Scholar 

  42. Elsayes, K.M., et al., Lipomatous adrenal metaplasia: computed tomography findings in 2 presumed cases. J Comput Assist Tomogr, 2009. 33(5): p. 715-6.

    Article  Google Scholar 

  43. Kawashima, A., et al., Imaging of nontraumatic hemorrhage of the adrenal gland. Radiographics, 1999. 19(4): p. 949-63.

    Article  CAS  Google Scholar 

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Supported by institutional CCSG (cancer center support grant) from the NIH/National Cancer Institute under Award Number P30CA016672.

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Correspondence to Corey T. Jensen.

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Morani, A.C., Jensen, C.T., Habra, M.A. et al. Adrenocortical hyperplasia: a review of clinical presentation and imaging. Abdom Radiol 45, 917–927 (2020). https://doi.org/10.1007/s00261-019-02048-6

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