Acth Independent Macronodular Adrenal Hyperplasia 2 C4014803
A rare condition where both adrenal glands develop multiple nodules that produce excess cortisol without ACTH stimulation, causing Cushing's syndrome. Adults are typically affected. Treatment may involve medications to block cortisol or surgical removal of the adrenal glands.
Key facts at a glance
- ICD-10
- E34.9
- ICD-11
- 5A00.0
- Body system
- endocrine
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Fatigue or weight changes
(endocrine)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Weight gain
(endocrine)Metabolic alkalosis
(endocrine)Acne
(endocrine)Striae
(endocrine)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Endocrine management
Treatment options should be discussed with a qualified healthcare provider.
Unilateral Adrenalectomy with Contralateral Partial Resection
Surgical approach combining removal of the dominant hyperplastic gland with partial resection or ablation of the contralateral gland to achieve hormonal control while preserving some adrenal function bilaterally.
Combination Medical Therapy
Use of multiple cortisol-lowering agents simultaneously to achieve better biochemical control while using lower doses of each individual drug, potentially reducing adverse effects. Common combinations include ketoconazole with metyrapone.
Additional treatment options
Laparoscopic Bilateral Adrenalectomy
Complete removal of both adrenal glands using minimally invasive techniques. Considered when medical therapy fails or quality of life is significantly impaired. Results in permanent adrenal insufficiency requiring lifelong replacement.
surgicalGlucocorticoid Receptor Antagonist Therapy
Mifepristone provides an alternative mechanism by blocking cortisol action at the receptor level rather than reducing production. Useful when cortisol synthesis inhibitors fail or are poorly tolerated.
medicationPsychological Support and Quality of Life Intervention
Comprehensive psychological care addressing depression, anxiety, cognitive changes, and body image issues common in Cushing syndrome. Includes cognitive behavioral therapy, support groups, and psychiatric medication management as indicated.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Acth Independent Macronodular Adrenal Hyperplasia 2 C4014803?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Nieman LK, Biller BM, Findling JW, et al. Treatment of Cushing's Syndrome: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2015.
中华医学会内分泌学分会. 促肾上腺皮质激素非依赖性肾上腺大结节增生症诊治专家共识. 中华内分泌代谢杂志. 2022.
Lacroix A, Bourdeau I, Lampron A, et al. Aberrant G-protein coupled receptor expression in cortisol-producing adrenal tumors. Horm Metab Res. 2010.
Patel D, Pham P, Rajaraman G, et al. Bilateral Adrenalectomy for ACTH-Independent Macronodular Adrenal Hyperplasia: A Systematic Review and Meta-Analysis. Ann Surg. 2021.
Medication Comparisons
Compare medications used to treat Acth Independent Macronodular Adrenal Hyperplasia 2 C4014803. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy