E24.3

Billable

Ectopic ACTH syndrome

HCC Category Mapping

V24HCC 23 — Other Significant Endocrine and Metabolic Disorders

0.230

ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders

0.000

RxHCCHCC 43 — Other Significant Endocrine and Metabolic Disorders

0.000

What This Code Means

Cushing's syndrome caused by a tumor outside the pituitary gland (such as in the lungs or pancreas) that produces ACTH hormone.

Coding Tips

•Document the location of the ectopic tumor; additional coding for the primary neoplasm may be required
•This is distinct from pituitary-dependent Cushing's disease

Clinical Significance

Ectopic adrenocorticotropic hormone syndrome occurs when a non-pituitary tumor produces adrenocorticotropic hormone autonomously, causing severe and often rapidly progressive hypercortisolism. The most common sources are small cell lung carcinoma, bronchial carcinoid tumors, and pancreatic neuroendocrine tumors, making this diagnosis an important marker for occult malignancy.

Documentation Requirements

✓Document the source tumor type and location, adrenocorticotropic hormone levels (typically very high), cortisol levels, imaging confirming the ectopic source, potassium levels (often severely hypokalemic), and management of both the tumor and cortisol excess.

Commonly Confused Codes

E24.0 (pituitary-dependent Cushing disease where the source is pituitary, not ectopic), E24.8 (other Cushing syndrome), D38.1 (neoplasm of uncertain behavior of trachea/bronchus which may be the source tumor).

Code Hierarchy

└E24Cushing's syndrome └E24.3Ectopic ACTH syndrome

└E24.3Ectopic ACTH syndrome