Diagnosis and management of adrenocorticotropic hormone-secreting pituitary carcinoma: a case report and review of the literature

Adrenocorticotropic hormone (ACTH)-secreting pituitary carcinomas (PC) are rare. The natural history and management of these carcinomas are poorly understood. We conducted a literature review using The MEDLINE database, including the search terms; ‘ACTH’ and ‘pituitary carcinoma’. We also describe in detail a case of ACTH-secreting PC. A total of 61 case reports were reviewed. Median age of diagnosis was 45 years (IQR: 34–54). Metastases to multiple organs were common (61%). Adjuvant therapy especially radiotherapy (78%), temozolomide (34%) and other medical therapy (29%) were frequently employed. The mortality was 53% with a median time to death from diagnosis of 1 year (IQR: 1–3). In conclusion, ACTH-secreting PC are associated with high mortality and a multidisciplinary team approach is recommended for optimal care due to the emerging modalities with possible efficacy.