Treatment of Carcinoid Tumor of Thymus in Cushing's Syndrome due to Ectopic ACTH Secretion
This protocol addresses thymic carcinoid presenting with Cushing's syndrome caused by ectopic adrenocorticotropic hormone (ACTH) secretion — the most frequent functional syndrome encountered in this setting. The clinical picture is driven by autonomous, tumour-derived ACTH excess leading to cortisol overproduction.
Clinical Approach
First-line management in this scenario involves a targeted pharmacological strategy using steroid synthesis inhibitors — the complete regimen, including agent selection and sequencing, is detailed in the full protocol.
References
DOI: 10.1016/j.annonc.2021.01.003
- CuS caused by ectopic ACTH secretion is the most frequent functional syndrome in ThC.
- 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].