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

Treatment of Pulmonary Neuroendocrine Tumor in Cushing’s Syndrome Caused by Ectopic ACTH Secretion

Pulmonary neuroendocrine tumors can drive autonomous secretion of adrenocorticotropic hormone (ACTH) from outside the pituitary, causing ectopic Cushing’s syndrome. This paraneoplastic complication significantly shapes the management approach and requires prompt, targeted intervention.

This protocol applies to patients with a pulmonary neuroendocrine tumor whose disease course is complicated by Cushing’s syndrome caused by ectopic ACTH secretion. The ectopic source drives cortisol excess, making control of hypercortisolism a central clinical priority alongside oncological management.

Management in this setting may involve early surgical intervention to address the cortisol excess at its source. Additional medical options exist for cases where surgery is not immediately feasible or sufficient.

Full regimen, sequencing, and complete options available in the structured protocol →
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].
  • In patients with refractory CuS, early bilateral adrenalectomy should be considered [IV, B].
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