Non-surgical treatment with octreotide for occult ectopic ACTH syndrome

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SUN 29-49-Congenital Adrenal Hyperplasia & Ectopic Cushing's
Sunday, June 16, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SUN-47
Seon A Kim*, Sun-Hee Ko and Bong-Yeon Cha
Seoul St. Mary’s Hospital, The Medical School of Catholic University, Seoul, South Korea
Background: Ectopic ACTH syndrome is a less frequent form of Cushing's disease. It may be associated with highly malignant tumors like small cell lung cancer but sometimes its focus of origin cannot be found.

Clinical case: A 55-year-old woman who had conditions of hypokalemic paralysis, uncontrolled diabetes mellitus, and pneumonia.

The results of her test were suggested ACTH dependent Cushing syndrome: elevated 24hr urinary free cortisol (77861.73 nmol/24hr, n<220 nmol/24hr), unsuppressed 1mg dexamethasone overnight test (cortisol after 1mg dex 2816.38 nmol/l, n<50 nmol/l), elevated serum cortisol (1846.59 nmol/l) and elevated plasma ACTH (1172.03 pg/ml). So she was supposed that she had Cushing syndrome due to ectopic ACTH producing tumor. An abdominal CT-scan & chest CT-scan showed bilateral adrenal hyperplasia and multifocal peripheral consolidations in the right upper lobe of lung, but no definite mass or lymph node enlargement. PET-CT showed the increased FDG uptake on both adrenal glands but no other site. We tried to confirm the existence of somatostatin receptor so we tested response to octreotide, somatostatin analogue. Plasma ACTH levels after Octreotide(50µg) subcutaneous injection resulted in significantly decreased plasma ACTH level. We measured basal plasma ACTH level(510.19 pg/ml), after 30min(281.73 pg/ml), 60 min(204.94 pg/ml), 90 min(172.3 pg/ml) and 120 min(313.76 pg/ml). In the next step we scanned the octreotide scintigraphy and SPECT but we couldn’t find it the increased uptake. We diagnosed occult ectopic ACTH syndrome and stopped the evaluation of it. But we confirmed octreotide response. So we tried treatment option with octreotide. We used octreotide 100mcg, every 8 hours, subcutaneous injection. The patient felt better and her anorexia decreased. After a week, the in follow-up test in the morning, plasma ACTH level was 150.19 pg/ml and serum cortisol level was 267.07 nmol/l. 24hr urinary free cortisol level was 128.01 nmol/24hr. The patient was well-trained about self-subcutaneous-injection because she had used insulin for herself. We found a potential of long-term treatment of octreotide.

Conclusion: Treatment option of occult ectopic ACTH syndrome is surgical adrenalectomy and medical treatment. We suggest that as an option of medical treatment, the octrotide for a long term should be used to well-compliant patient who has the ectopic ACTH syndrome.

Nothing to Disclose: SAK, SHK, BYC

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