SPONTANEOUS REMISSION OF ECTOPIC CUSHINGS-SYNDROME DUE TO PHEOCHROMOCYTOMA - A CASE-REPORT

A young female adult with Cushing's syndrome arising from ectopic prod uction of corticotropin (ACTH) from an adrenal pheochromocytoma showed spontaneous clinical and biochemical remission of hypercortisolism af ter a brief period of ketoconazole administration. Despite continued r emission of the hypercortisolism over the next 18 months, there was pr ogressive catecholamine hypersecretion with significant morbidity as a result of the pheochromocytoma. Surgical resection of the left adrena l gland revealed a pheochromocytoma showing focal cytoplasmic immunost aining for ACTH and marked diffuse compact cell hyperplasia in the adr enocortical tissue. To our knowledge this is the first reported case o f spontaneous clinical and biochemical remission of ectopic ACTH produ ction from a pheochromocytoma. The pathogenesis of the remission remai ns unknown.