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- Title
Characteristics and outcomes of the Finnish ectopic ACTH syndrome cohort.
- Authors
Toivanen, Sanna; Leijon, Helena; Arola, Aura; Soinio, Minna; Hämäläinen, Päivi O.; Metso, Saara; Knutar, Otto; Koivikko, Minna; Ebeling, Tapani; Moilanen, Leena; Norvio, Leena; Tamminen, Marjo; Rautiainen, Päivi; Vehkavaara, Satu; Ryhänen, Eeva; Pekkarinen, Tuula; Matikainen, Niina; Sane, Timo; Schalin-Jäntti, Camilla
- Abstract
Purpose: Ectopic ACTH syndrome (EAS) is rare. We established a national cohort to increase awareness and address unmet needs. Methods: The 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). Results: The 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. Conclusions: NET/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.
- Publication
Endocrine (1355008X), 2021, Vol 74, Issue 2, p387
- ISSN
1355-008X
- Publication type
Article
- DOI
10.1007/s12020-021-02768-0