ArticleEndocrine2021
Characteristics and outcomes of the Finnish ectopic ACTH syndrome cohort.
Article in Endocrine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 20 citations in OpenAlex.
- Urinary-free cortisol-based thresholds for differentiating ACTH-dependent Cushing: a Spanish validation study.The Journal of clinical endocrinology and metabolism · 2026Article
- Fournier gangrene in a patient with corticotrophinoma and diabetes treated with sodium-glucose cotransporter 2 inhibitor.JCEM case reports · 2026Article
- European Society of Neuroendocrine Tumors (ENETS) 2025 guidance paper for lung and thymic carcinoids.Journal of neuroendocrinology · 2026Article
- Surgical management of ectopic Cushing's syndrome from a pulmonary carcinoid secreting CRH supported by intraoperative ACTH monitoring - a case report.Frontiers in endocrinology · 2026Article
- Long-term outcomes and survival predictors in patients with ectopic ACTH syndrome: data from a retrospective cohort study.Endocrine connections · 2025Article
- Bilateral Adrenal Ablation for the Treatment of Ectopic ACTH Syndrome With Severe Hypokalemia: A Case Report and a Literature Review.Clinical case reports · 2025Article
- Unusual infections and thrombotic events in Cushing's syndrome.Journal of endocrinological investigation · 2025Review
- Transcriptome-Wide Analysis of Pituitary and Ectopic Adrenocorticotropic Hormone-Secreting Tumors.Cancers · 2025Article
- Differentiating lung neuroendocrine neoplasms from tumor-like infection using CT in patients with ectopic ACTH syndrome.Insights into imaging · 2024Article
- Article
- Effect of mitotane in patients with ectopic adrenocorticotropic hormone syndrome caused by advanced pancreatic neuroendocrine tumors: a case series and review of the literature.The Journal of international medical research · 2024Review
- Management of Functional Pancreatic Neuroendocrine Neoplasms.Current treatment options in oncology · 2023Review
- Paraneoplastic isolated adrenocorticotropic hormone deficiency revealed after immune checkpoint inhibitors therapy: new insights into anti-corticotroph antibody.Frontiers in immunology · 2023Article
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Authors and funding
19 authors at 9 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeEctopic ACTH syndrome (EAS) is rare. We established a national cohort to increase awareness and address unmet needs.
methodsThe Finnish national EAS cohort includes 60 patients diagnosed in 1997-2016. We assessed clinical features, diagnostic work-ups, treatments, incidence, and outcomes of subgroups occult tumor (OT), well-differentiated neuroendocrine tumor G1/G2 (NETG1/G2) and NET G3/neuroendocrine carcinoma (NETG3/NEC).
resultsThe distribution of OT, NETG1/G2, and NETG3/NEC was 10 (17%), 20 (33%), and 30 (50%), respectively; and median follow-up 22 months (0-249). Annual incidence (0.20-0.93 per million inhabitants) and tumor subgroups (OT vs. NEC) varied across the country. The longest diagnostic delay from EAS onset to radiological tumor identification was 48 months. In NET/NEC, 6/50 (12%) were diagnosed 1-24 years before EAS onset. Osteoporotic fractures (32%) and severe infections (55%) were common. The CRH stimulation test accurately diagnosed EAS in 25/31 (81%). Metyrapone (≤6 g daily, prescribed in 88%) was well tolerated. In NETG1/G2, 13/20 (65%) underwent curative resection of the primary tumor; four experienced recurrence within 2-12 years. In OT, 70% underwent bilateral adrenalectomy. Five-year overall survival in OT, NETG1/G2, and NETG3/NEC was 90%, 55%, and 0%, respectively (P < 0.001). Morning cortisol, hypokalemia, infections, metastatic disease, and acute onset were negative, whereas resection of the primary tumor and bilateral adrenalectomy were positive predictors of survival.
conclusionsNET/NEC may precede EAS onset by several years. In NETG1/G2, recurrences may occur > 10 years after successful primary surgery. Tumor subgroup (OT, NETG1/G2, NEC) was an independent predictor of survival.
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