Multiple hormone secretion may indicate worse prognosis in patients with ectopic Cushing's syndrome
PURPOSE: Ectopic Cushing's syndrome (ECS) caused by an ACTH secreting neuroendocrine neoplasm (NEN) is a rare and challenging condition. We aimed to detect predictive and prognostic parameters for ECS patients identified from a retrospective, comprehensive cohort of NENs treated at a tertiary referral center.
METHODS: Medical records of 886 patients with NENs were reviewed. We identified 51 patients with ECS (33 females/18 men); mean age 52 ± 15 years (SD). Clinical parameters including symptoms, biochemical markers, and survival were extracted and further analyzed.
RESULTS: The primary tumor was located in the thorax (n = 28) or pancreas (n = 15) or was of unknown primary origin (n = 8). In 30 patients, tumor and ECS were diagnosed simultaneously. In 12 patients, the NEN diagnosis preceded ECS development, with a median time of 43.5 months (range: 9-96), and 10 of these showed radiological tumor progression at ECS diagnosis. Twenty-one patients had multiple hormone secretion, which correlated with shorter overall survival (OS), p = 0.012 (HR 2.4 (95% CI 1.2-4.9)), as did high morning cortisol, p = 0.037 (HR 2.3 (1.0-5.2)), higher tumor grade, p = 0.044 (HR 2.3 (1.0-5.1)), and diabetes, p = 0.050 (HR 2.4 (1.0-6.0)).
CONCLUSIONS: Multiple hormone secretion, high morning cortisol, higher tumor grade, and diabetes were correlated with shorter OS. Development of ECS in patients with a non-functioning NEN may indicate tumor progression. Multiple hormone secretion should be considered as a bad prognostic sign in ECS patients and should lead to intensified clinical management.
Full Text Links
Find Full Text Links for this Article
You are not logged in. Sign Up or Log In to join the discussion.