Pulmonary neuroendocrine tumor
ICD-10 C34.9 · ICD-11 2C25.4

Pulmonary Neuroendocrine Tumor with Cushing's Syndrome Caused by Ectopic ACTH Secretion

In a subset of patients with pulmonary neuroendocrine tumor, the tumor autonomously secretes adrenocorticotropic hormone (ACTH), driving a distinct and clinically significant complication that requires targeted management alongside the underlying malignancy.

Clinical Scenario

This protocol addresses pulmonary neuroendocrine tumor complicated by Cushing's syndrome caused by ectopic ACTH secretion — a paraneoplastic state in which excess cortisol production arises from tumor-driven ACTH release rather than from a pituitary or adrenal source.

Treatment Approach (partial overview)

The treatment of choice in this setting involves steroid synthesis inhibitors to control cortisol excess. The specific agents, their combination, and the full clinical algorithm are available in the complete protocol.

Full regimen, sequencing, and decision criteria are in the structured protocol below.

References
DOI: 10.1016/j.annonc.2021.01.003
The treatment of choice for CuS by ectopic secretion of ACTH includes steroid synthesis inhibitors such as metyrapone and/or ketoconazole [IV, B].
Metyrapone and/or ketoconazole are recommended as first-line therapy for CuS [IV, B].
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