The Human Adrenal Cortex

1. Introduction.- 2. Historical Aspects.- A. The Normal Adrenal Cortex.- 3. Origin and Development of the Adrenal Gland.- Embryology.- Fetal Development.- Postnatal Development.- 4. Structure of the Adult Adrenal Cortex.- Gross Anatomy.- Weight.- Blood Supply.- Light-Microscopic and Ultrastructural Appearance.- The Normal Gland.- The Effects of Adrenocorticotrophic Hormone (ACTH).- Intraglandular Vascularisation.- Lymphatic Supply.- Innervation.- 5. The Effect of Stress on the Structure of the Adrenal Cortex.- 6. Morphological Changes in the Adrenal Cortex with Age.- The Prenatal Cortex.- The Neonatal and Prepubertal Cortex.- The Postpubertal Cortex.- Adrenocortical Nodules.- The Autopsy ‘Black’ Nodule.- 7. Iatrogenic Changes.- ACTH.- Steroid Therapy.- Inhibitors of Adrenal Steroidogenesis.- Aminoglutethimide.- Spironolactone.- O,p’-DDD (Mitotane, Lysodren).- Other Inhibitors.- 8. Functional Activity of the Adrenal Cortex.- Adrenal Chemistry.- Steroids.- Lipids.- Vitamins.- Hormonogenesis by the Adult Adrenal Cortex.- Pathways of Steroidogenesis.- Molecular Mechanisms of Steroidogenesis.- Inhibitors of Steroidogenesis.- Control of Adult Steroidogenesis.- Glucocorticoids.- Mineralocorticoids.- Sex Steroids.- Hormonogenesis by the Prepubertal and Pubertal Cortex.- Hormonogenesis by the Prenatal Adrenal Cortex.- Pathways of Steroidogenesis.- The Feto-placental Unit.- Functions of Fetal Adrenal Steroids.- Control of Prenatal Hormonogenesis.- 9. Regulation of Growth of the Adrenal Cortex.- The Prenatal Gland.- Growth.- Involution.- The Postnatal Gland.- Adult Tissue Homeostasis and Regenerative Growth.- 10. Functional Zonation of the Adrenal Cortex.- Zonal Functions of the Cortex.- Mechanisms of Functional Zonation.- B. Hypercorticalism.- 11. General Incidence and Pathological Changes.- Factors Predisposing to Hypercorticalism.- 12. Cushing’s Syndrome.- Bilateral Adrenocortical Hyperplasia.- Simple (or Diffuse) Hyperplasia.- Bilateral Nodular Hyperplasia.- Hyperplasia and the ‘Ectopic ACTH’ Syndrome.- Congenital and Familial ‘Idiopathic’ Cushing’s Syndrome with Hyperplasia.- Infantile Onset.- Pubertal/Adult Onset.- Adrenocortical Tumours.- Adenomas.- Carcinomas.- The Attached and Contralateral Adrenal Gland.- Prognosis.- 13. The Adrenogenital Syndrome and Related Conditions.- Congenital Adrenocortical Hyperplasia (CAH).- CAH With Virilism.- 21-Hydroxylase Deficiency...- 11 ß-Hydroxylase Deficiency.- ?5-3ß-Hydroxysteroid Dehydrogenase-isomerase Deficiency.- Pathology of CAH With Virilism.- Non-virilising CAH Syndromes and Related Defects...- 17-Hydroxylase Deficiency.- Defective Cholesterol Utilisation.- Defective Aldosterone and 18-hydroxycorticosterone.- Biosynthesis (Corticosterone Methyl Oxidase Type I and Type II Deficiencies).- C17–20 Desmolase (Lyase) Deficiency.- Pathology of CAH Without Virilism.- Genetics of CAH.- Virilising Adrenocortical Tumours.- Incidence and Symptoms.- Functional Properties.- Pathology.- Prognosis.- Adrenal Tumours Causing Feminisation.- Incidence and Symptoms.- Functional Properties.- Pathology.- Prognosis.- Testicular Changes.- Attached and Contralateral Adrenal Gland in the.- Adrenogenital Syndrome With Tumours.- Non-tumorous Adrenal Feminisation.- 14. The Distinction Between Benign and Malignant Adrenocortical Tumours Causing Cushing’s and/or the Adrenogenital Syndromes: Structural and Functional Criteria.- Morphology.- Function.- Autonomy.- Aetiology of the Abnormal Steroidogenesis.- Conclusions.- 15. Hyperaldosteronism and Related Syndromes of Mineralocorticoid Excess.- Hyperaldosteronism With Low Plasma Renin (Primary Aldosteronism).- Symptoms and Incidence.- Pathology.- Incidence of Pathological Adrenal Changes in Primary Aldosteronism.- Adrenocortical Tumours.- Prognosis.- The Attached and Contralateral Adrenal Gland in Tumorous Primary Aldosteronism.- Non-tumorous Hyperaldosteronism With Low Plasma Renin.- Hyperaldosteronism With High Plasma Renin (Secondary Aldosteronism).- Bartter’s Syndrome (‘Tertiary’ Aldosteronism).- Syndromes of Mineralocorticoid Excess Without Hyperaldosteronism.- Congenital.- Acquired.- Non-tumorous.- Adrenocortical Tumours.- Aetiology and Correlation of Structure and Function in Conn’s Syndrome.- Tumour Structure and Function.- Structure and Function of the Attached Gland.- The Relationship of Nodules and Adenomas in Conn’s Syndrome: a Possible Functional Continuity?.- 16. ‘Non-functioning’ (Non-hormonal) Adrenocortical Tumours.- Incidence and Symptoms.- Functional Activity.- Pathology.- Prognosis.- The Attached and Contralateral Gland in Non-hormonal Adrenocortical Tumours.- C. Hypocorticalism.- 17. Acute Acquired Adrenoprivic Hypocorticalism.- 18. Chronic Acquired Adrenoprivic Hypocorticalism (Addison’s Disease).- Organ-specific Autoimmune Adrenalitis (Idiopathic Addison’s Disease).- Pathology.- Aetiology.- Adrenoleukodystrophy (Addison-Schilder’s Disease).- Incidence and Symptoms.- Pathology.- Adrenal Cortex.- Other Tissues.- Aetiology of Adrenal Changes in Adrenoleukodystrophy.- Tuberculosis of the Adrenal Gland.- Pathology.- Amyloidosis.- Pathology.- Metastatic Carcinoma.- Miscellaneous Causes Including Non-tuberculous Infections.- 19. Congenital Adrenoprivic Hypocorticalism.- Idiopathic Congenital Adrenocortical Hypoplasia and the Syndrome of ACTH Unresponsiveness.- Incidence and Symptoms.- Pathology.- Congenital Adrenal Hyperplasia.- 20. Tropoprivic Hypocorticalism.- Acquired.- Iatrogenic.- Pituitary-derived.- Congenital.- Incidence.- Pathology.- D. Other Pathological Changes.- 21. Haemorrhage and Necrosis.- Incidence.- Signs and Symptoms.- Pathology.- Aetiology.- Prognosis.- 22. Congenital Defects of Adrenal Development.- Malformations and Heterotopias.- Adrenal Rests and Accessory Adrenal Glands.- Incidence and Distribution.- Hyperplasia and Tumours of Accessory Adrenocortical Tissue.- Ectopic Tissues Within the Adrenal Cortex.- Gonadal Cells.- Other Tissues.- Congenital Adrenocortical Cytomegaly.- ‘Idiopathic’ Congenital Cytomegaly.- Cytomegaly in the Beckwith-Weidemann Syndrome...- 23. Myelolipoma and Related Changes.- Incidence and Symptoms.- Pathology.- Aetiology.- 24. Cysts.- Incidence and Symptoms.- Pathology.- 25. Miscellaneous Changes.- Erythroblastosis Fetalis.- Haemochromatosis.- Syphilis Î.- Malacoplakia.- Wolman’s Disease.- Post-irradiation Fibrosis.- Appendix A. Plasma Steroids.- Appendix B. Urinary Steroids.- References.