Adrenal disease

Survival probabilities in patients with ectopic Cushing's syndrome—a systematic review and a single-arm meta-analysis

Eur J EndocrinolPublished Systematic review and single-arm meta-analysis

Authors' conclusion

Survival in ECS varies considerably, mainly due to the underlying origin of the tumor, tumor stage, and severity of the hypercortisolism. Further studies analyzing the importance of factors affecting survival are needed.

Study context

Population
Patients with ectopic Cushing's syndrome (ECS); PubMed, Scopus and Web of Science searched up to October 18, 2023
Participants
1,148 patients40 studies
Comparison
Pulmonary Neuroendocrine Neoplasm (NEN) vs Occult ECS vs Thymic NEN vs Pancreatic NEN
Outcome
Survival in patients with ECS
Follow-up
1 and 5 years
Methodological detailsData extraction and risk of bias assessment by 3 independent investigators
  • Data extraction and risk of bias assessment by 3 independent investigators

Pooled result

As reported in the abstract

Pooled mean survival probability, ECS of mixed etiologies

1-year survival probability

78%

5-year survival probability

47%

Further pooled results

5-year survival probability by ECS form

Factors influencing survival

Studies reporting factors influencing survival
8
Total resection of the primary tumor associated with better overall survival
Unresectable tumors, metastatic disease at diagnosis, severe hypercortisolism, hypokalemia and new-onset DM associated with worse prognosis

Original publication

Survival probabilities in patients with ectopic Cushing's syndrome—a systematic review and a single-arm meta-analysis. Eur J Endocrinol. Published 2 June 2025. doi:10.1093/ejendo/lvaf114

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