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

Ieva Lase (Corresponding Author), Ieva Strele, Malin Grönberg, Gordana Kozlovacki, Staffan Welin, Eva Tiensuu Janson

    Research output: Contribution to journalArticlepeer-review

    10 Citations (Scopus)
    14 Downloads (Pure)

    Abstract

    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.

    Original languageEnglish
    Pages (from-to)351-360
    Number of pages10
    JournalHormones
    Volume19
    Issue number3
    DOIs
    Publication statusPublished - 16 Jan 2020

    Keywords*

    • ACTH
    • Ectopic Cushing’s syndrome
    • Hypercortisolism
    • Hypokalemia
    • Multiple hormone secretion
    • Neuroendocrine

    Field of Science*

    • 3.2 Clinical medicine
    • 3.3 Health sciences

    Publication Type*

    • 1.1. Scientific article indexed in Web of Science and/or Scopus database

    Fingerprint

    Dive into the research topics of 'Multiple hormone secretion may indicate worse prognosis in patients with ectopic Cushing’s syndrome'. Together they form a unique fingerprint.

    Cite this