Kc. Loh et al., SPONTANEOUS REMISSION OF ECTOPIC CUSHINGS-SYNDROME DUE TO PHEOCHROMOCYTOMA - A CASE-REPORT, European journal of endocrinology, 135(4), 1996, pp. 440-443
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.