Related Experiment Video
Updated: Aug 7, 2025
A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
Adrenal Medullary Hyperplasia: A Systematic Review and Meta-analysis
Rafal Ganni1, David J Torpy2,3, Henrik Falhammar4,5
1School of Medicine, Sydney, The University of Notre Dame, Darlinghurst, NSW 2010, Australia.
Context:
Adrenal medullary hyperplasia (AMH) is a rare, incompletely described disorder of the adrenal medulla that is associated with catecholamine excess.
Objective:
To increase knowledge about AMH by reviewing the reported cases of this disorder.
Design:
Systematic review and meta-analysis of the genotype/phenotype relationship in all reported cases of AMH.
Setting:
Literature review and analysis.
Patients Or Other Participants:
All cases of AMH published to date.
Main Outcome Measure(S):
Characteristics of AMH cases and genotype-phenotype relationships.
Results:
A total of 66 patients, median age of 48 years, were identified from 29 reports. More than one-half were male (n = 39, 59%). The majority had unilateral (73%, n = 48) disease; 71% (n = 47) were sporadic and 23% (n = 15) were associated with the MEN2. Most (91%, n = 60) displayed signs and symptoms of excess catecholamine secretion, particularly hypertension. Elevated catecholamine concentrations (86%, n = 57) and adrenal abnormalities on imaging were common (80%, n = 53). More than one-half (58%, n = 38) had concurrent tumors: pheochromocytoma (42%, n = 16/38); medullary thyroid cancer (24%, n = 9/38); and adrenocortical adenoma (29%, n = 11/38). Most (88%, n = 58) underwent adrenalectomy with 45/58 achieving symptom resolution. Adrenalectomy was less common in patients under 40 years and those with bilateral disease (both P < .05).
Conclusion:
AMH may be sporadic or associated with MEN2, most have catecholamine excess and imaging abnormalities. Unilateral involvement is more common. Most reported patients have been treated with adrenalectomy, which is usually curative with regard to catecholamine hypersecretion.
Related Concept Videos
01:27Adrenal Gland Disorders
Adrenal insufficiency, characterized by insufficient cortisol and aldosterone production, leads to conditions like Addison's disease. This disorder, affecting the adrenal cortex, exhibits symptoms such as skin bronzing, dehydration, low blood pressure, fatigue, and weight loss. Congenital adrenal hyperplasia, a genetic ailment causing...
01:31Hormones of the Adrenal Glands
The adrenal cortex, a powerhouse of hormone synthesis, generates over two dozen corticosteroid hormones. The zona glomerulosa produces mineralocorticoids, exemplified by aldosterone, influencing the electrolyte composition of body fluids. The synthesis of glucocorticoids such as cortisol and...
01:17Anatomy of the Adrenal Glands
These glands possess a distinctive yellow tinge due to the stored cholesterol and fatty acids required for hormone synthesis. They are encased in a fibrous capsule and cushioned by fat.
The adrenal gland comprises two distinct...
01:27Sympathetic Pathways: Collateral Ganglia and Adrenal Medulla
Collateral Ganglia
Sympathetic preganglionic axons reach the collateral ganglia along the route of splanchnic nerves. These nerves bypass the sympathetic trunk and communicate with sympathetic postganglionic neurons housed in the prevertebral ganglia. These ganglia supply the organs of the abdominopelvic cavity.
The greater splanchnic nerve, formed by the...
01:37Hypothalamic-Pituitary Axis
01:21Smooth Endoplasmic Reticulum
The ER provides optimal conditions for synthesizing steroid hormones and lipids, such as phospholipids and triglycerides. Traditionally, lipid metabolism was considered to be a smooth ER function. However, there is no direct evidence to prove that rough ER is completely excluded from lipid...