Introduction. Ectopic adrenocorticotropic hormone (ACTH) syndrome is very rare endocrine disorder caused by excess secretion of ACTH by non-pitutary tumor. Prolonged glucocorticoid excess causes Cushing’s syndrome, associated with complications, increasing mortality. First choice treatment is definitive tumor resection. Main issue in diagnosis is to find ectopic ACTH secretion source. For patients with occult or metastatic ectopic ACTH secretion adrenal steroidogenesis inhibitors are recommended as first line therapy to control hypercortisolemia.
Case presentation. We are presenting unique cases when ectopic ACTH secretion source was not found. The first patient was treated with etomidate for 10 months, because other treatment options were ineffective or unavailable. After 2 years he received palliative chemotherapy and octreotide because of disease progression. Currently disease stabilization is achieved. For the second patient Cushing syndrome was diagnosed during hospitalization due to sepsis at intensive care unit. The origin of ectopic ACTH secretion was confirmed by diagnostic tests. Metyrapone was prescribed for hypercortisolemia correction, but resistant hypertension, proteinuria influenced the deterioration of kidney function. Last episode of sepsis was complicated by septic shock, multiple organ dysfunction syndrome. Hemodialysis was started. Very high ACTH concentration decreased and caused adrenal insufficiency, the hydrocortisone was administered for replacement therapy.
Conclusions. Despite of modern diagnostic exams ectopic ACTH secreting tumor not always may be found. Consequently definitive surgical treatment is not available. Diagnostic possibilities, pharmacologic management and clinical outcomes are reviewed in the article.
