A CASE OF OCCULT ECTOPIC CORTICOTROPIN (ACTH) SYNDROME MAITAINING REMISSION STATE OVER 4 YEARS AFTER BILATERAL ADRENALECTOMY

Abstract

A long-lasting state of remission of over 4 years of occult ectopic cor- ticotropin (ACTH) syndrome in a 68-year-old female is reported. She presented with general malaise, muscle atrophy and weakness of extremities, general petechiae and hypokalemia. Although ectopic ACTH syndrome was suspected due to marked hypercor- tisolism and ACTH elevation, the source of ACTH secretion could not detected by any diagnostic technique. Because of that effective suppression of plasma cortisol levels by torilostane, a 3 β-hydroxy-steroid-dehydrogenase inhibitor, could not obtained, so bilateral adrenalectomy was performed. After the operation, supplemental therapy of hydrocor- tisone and Florinef, 9 α-fluorohydrocortisone, was initiated and the plasma ACTH levels decreased gradually. At the 6th postoperative month, the levels of ACTH remained within the normal range and her general status markedly improved. At the 4th postoperative year, the plasma ACTH still maintained normal levels with no recurrence. Cases of occult ectopic ACTH syndrome which remain in remission for a long period after bilateral adrenalectomy are rare and interesting. Bilateral adrenalectomy may be the first choice of treatment for occult ectopic ACTH syndrome with the support of chemical therapy.