ECTOPIC ACTH-SECRETING TUMORS - A HETEROGENOUS ENTITY

OBJECTIVES: ACTH-secreting non-pituitary tumors are a rare cause of Gu shing's disease. We report the clinical course, prognostic aspects and molecular analysis data in three patients for whom the diagnosis was confirmed but who had variable clinical features and laboratory result s. CASE REPORTS: Patient n degrees 1 had severe hypercorticism which r apidly progressed to death 13 months after diagnosis. in patient n deg rees 2, signs of hypercorticism severe, leading to death 5 years after discovery of the causal carcinoid tumor. Patient n degrees 3 had mode rate hypercorticism and has survived for more than 25 years, DISCUSSIO N: These 3 ectopic tumors are representative examples of a wide range of possible ACTH-secreting ectopic tumors. in highly malignant poorly- differentiate tumors such as small-cell anaplastic carcinomas, ACTH pr oduction is aberrant and poorly controlled, and thus not particularly effective. At the other extreme, typical benign bronchial carcinomas h ave a high degree Of neuroendocrine differentiation and secrete ACTH i n a well-controlled manner difficult to distinguish from corticotropic adenomas, further exaggerating the diagnostic pitfalls.