Endocrine Abstracts

Background: Ectopic ACTH syndrome (EAS) is rare and published series are scarce.

Aim: To increase the understanding of EAS we describe 31 consecutive cases in years 1997–2016.

Results: Of the 31 patients, 65% (20/31) were women and 35% (11/31) men. Median follow-up was 23 months (0–217). Median age at diagnosis was 62 years (21–78), with peak incidences in age groups 30–39 and 60–69 years. Most patients presented with hypokalemia (87%), muscle weakness (77%), ankle edema (74%), hypertension (71%), psychiatric manifestations (58%), changes in body fat distribution (58%), and bruises (51%). Median (range) cortisol, ACTH and 24 h urinary free cortisol were 1467 nmol/l (121–4380), 155 ng/l (16–4700), 8221 nmol/l (568–69038), and median (range) cortisol after 1 mg DST 1103 nmol/l (74–3390), respectively. Thirteen (42%) underwent CRH-testing, which was accurate (< 50% increase in ACTH and < 20% in cortisol) in 9/13 (69%) patients. Underlying tumours: pancreatic NET (n=6), pulmonary NET (n=6), pulmonary small cell carcinoma (n=6), unknown primary (n=4; no tumour detected n=2, disseminated disease n=2), medullary thyroid carcinoma (n=3), thymic NET (n=2), and, ileum NET, prostate carcinoma, poorly differentiated pulmonary NEC, poorly differentiated pulmonary LCC (n=1 for each). Median overall survival was 23 month (0–217). At the end of follow-up, 12/31 (39%) patients were alive. Four patients initially cured recurred at 2, 6, 6 and 10, and 12 years, respectively. Surgery of the primary tumour was possible in 10/31 (32%) patients, nine (90%) of whom were alive at the end of follow-up. Bilateral adrenalectomy was performed in 10/31 (32%) patients, three (30%) of whom were alive at the end of follow-up. Outcome was best in thymic and pulmonary NETs.

Conclusion: EAS affects women in 65% of cases. Incidence peaks in age groups 30–39 and 60–69 years. Recurrences are possible > 10 years later. Overall survival is poor.