Ectopic ACTH Syndrome

A wide range of tumors has been described to produce biologically active ACTH. Many studies have shown that the molecular mechanism of this syndrome is due mainly to a precursor of the ACTH molecule which is proopiomelanocortin (POMC) and is a 31 kD glycoprotein. These precursor molecules, which are produced normally by the normal cells, are produced by some tumors in greater concentrations. Some tumors convert these precursors to biologically active ACTH, thus causing this paraneoplastic syndrome [4], The ectopic ACTH syndrome differs from the classical Cushing's disease by several markers: (1) serum and urine Cortisol concentrations are usually markedly increased; (2) plasma ACTH and POMC levels are usually markedly increased and the ratio of POMC to ACTH concentration is high; (3) Hypokalemia is common; and (4) Dexamethasone does not suppress ACTH and Cortisol levels.

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