Clinicopathologic features, treatments, and survival of patients with ectopic Cushing's syndrome from neuroendocrine tumours: data from an Italian multicentre study

Davì, Maria Vittoria; Cosaro, Elisa; Piacentini, Serena; Reimondo, Giuseppe; Albiger, Nora; Arnaldi, Giorgio; Faggiano, Antongiulio; Mantovani, Giovanna; Fazio, Nicola; Grimaldi, Franco; Mannelli, Massimo; Giraldi, Francesca Pecori; Martini, Chiara; Ferone, Diego; Campana, Davide; Scaroni, Carla; Terzolo, Massimo; DeMarinis, Laura; Francia, Giuseppe

doi:10.1530/endoabs.37.gp.28.01

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“…Because of the rarity of ECS, most of the publications are clinical case reports. In the case series published [20][21][22][23][24][25][26][27][28][29][30][31][32][33][34], the number of included patients is usually less than 50. Only two centers have published studies of > 100 patients with ECS [23,35].…”

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confidence: 99%

Multiple hormone secretion may indicate worse prognosis in patients with ectopic Cushing’s syndrome

et al. 2020

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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.

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