Acth Independent Macronodular Adrenal Hyperplasia 1 C1857451
A rare genetic condition causing both adrenal glands to enlarge and overproduce cortisol. Present from birth. Treatment usually involves medication to control hormone levels, with surgery considered in some cases.
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
Hypertension
(endocrine)Hypokalemia
(endocrine)Hyperglycemia
(endocrine)Moon face
(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
Surgical removal of the larger or more active adrenal gland in macronodular hyperplasia may control cortisol excess when asymmetric involvement exists. Minimally invasive approaches are preferred when anatomically feasible.
Cortisol Synthesis Inhibition
Pharmacological inhibition of adrenal cortisol production using agents such as ketoconazole, metyrapone, or etomidate in severe cases. Often requires combination therapy for adequate cortisol control.
Additional treatment options
Bilateral Adrenal-Sparing Surgery
Subtotal adrenalectomy removing hyperplastic tissue while preserving some adrenal function, potentially avoiding permanent adrenal insufficiency. Reserved for carefully selected patients with severe disease unresponsive to medical therapy.
surgicalPercutaneous Adrenal Ablation
Image-guided chemical or thermal ablation of hyperplastic adrenal tissue as a minimally invasive alternative for patients who are poor surgical candidates. May require multiple sessions for adequate treatment.
procedureSerial Imaging and Biochemical Surveillance
Regular monitoring of adrenal gland size, nodule characteristics, and cortisol production through biochemical testing and imaging studies. Allows early identification of disease progression or inadequate response to therapy.
monitoringWhen 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 1 C1857451?
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.
Endocrine Society Writing Committee. Treatment of ACTH-independent macronodular adrenal hyperplasia: Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2021.
Lacroix A, Bourdeau I, Bourlier M. Pathophysiology and management of ACTH-independent macronodular adrenal hyperplasia. Endocr Rev. 2020.
Nomura R, Tanaka K, Yoshida S, et al. Unilateral adrenalectomy for ACTH-independent macronodular adrenal hyperplasia: a multicenter prospective study. Surgery. 2019.
Wang J, Chen L, Liu Q, et al. Comparative effectiveness of surgical approaches for ACTH-independent macronodular adrenal hyperplasia: systematic review and meta-analysis. Eur J Endocrinol. 2022.
Medication Comparisons
Compare medications used to treat Acth Independent Macronodular Adrenal Hyperplasia 1 C1857451. 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy