ICD-10 Code E24.3 – Ectopic ACTH syndrome (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Ectopic ACTH syndrome is E24.3.
2026 ICD-10-CM Diagnosis Code E24.3 – Ectopic ACTH syndrome

What it is

Ectopic ACTH syndrome is a form of Cushing syndrome caused by a non-pituitary tumor producing ACTH. It leads to excess cortisol from an abnormal hormone source outside the pituitary.

Clinical signs

Common findings include features of hypercortisolism such as central weight gain, muscle weakness, hypertension, and glucose intolerance. Clinical features vary; refer to documentation.

When to use this code

Use E24.3 when documentation states ectopic ACTH production, ectopic ACTH syndrome, or Cushing syndrome due to an ACTH-secreting tumor outside the pituitary. Confirm the provider identifies the cause as ectopic, not pituitary or exogenous.

Do not use for

Do not use this code for pituitary-dependent Cushing disease, adrenal cortisol excess, or Cushing syndrome from steroid use. Check documentation when the source of ACTH is not specified.

Coding tip

Code E24.3 only when the record clearly links hypercortisolism to ectopic ACTH secretion.

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