Ectopic ACTH syndrome (EAS) is rare but is frequently a severe condition because of the intensity of the
hypercortisolism that may be dissociated from the tumoral condition. EAS should often be considered as an endocrine emergency requiring an emergency response both in terms of diagnostic procedures and therapeutic interventions. Patient management is complex and necessitates dual skills, in the diagnosis and treatment of CS and in the specific management of
neuroendocrine tumors (NET). Therefore, initial management should be performed ideally by experienced endocrinology teams in collaboration with specialized hormonal laboratory, modern imaging platforms and intensive care units. Diagnostic procedures vary according to the endocrine and tumoral contexts but should be reduced to a minimum in intense
hypercortisolism. Preventive and curative treatments of
cortisol-induced comorbidities, non-specific management of
hypercortisolism and etiological treatments should be considered simultaneously. Therapeutic strategies vary according to (1.) the intensity of
hypercortisolism, the general condition of the patient and associated comorbidities and (2.) the tumoral status, ranging from resectable
ACTH secreting
tumors to non-resectable metastatic endocrine
tumors or occult
tumors. The ideal treatment is complete excision of the
ACTH-secreting
tumor that can be performed rapidly or after preoperative preparation using
cortisol-lowering drugs. When this is not possible, the therapeutic strategy should be discussed by a multidisciplinary experienced team in a personalized perspective and include variable combinations of pharmacological agents, bilateral
adrenalectomy and non-specific tumoral interventions. Here we discuss the diagnosis and therapeutic strategies including the modern, currently available tools and emphasize on the operational effectiveness of care.