Characteristics and outcomes of the Finnish ectopic ACTH syndrome cohort.

Autor: Toivanen S; Endocrinology, Abdominal Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.; Divison of Endocrinology, Helsinki University Hospital, Hyvinkää, Finland., Leijon H; Department of Pathology, University of Helsinki and HUSLAB, Helsinki University Hospital, Helsinki, Finland., Arola A; Department of Endocrinology, Turku University Hospital and University of Turku, Turku, Finland., Soinio M; Department of Endocrinology, Turku University Hospital and University of Turku, Turku, Finland., Hämäläinen PO; Department of Internal Medicine and Tampere University, Faculty of Medicine and Health Technology, Tampere University Hospital, Tampere, Finland., Metso S; Department of Internal Medicine and Tampere University, Faculty of Medicine and Health Technology, Tampere University Hospital, Tampere, Finland., Knutar O; Department of Internal Medicine, Vaasa Central Hospital, Vaasa, Finland., Koivikko M; Department of Internal Medicine, Oulu University Hospital and University of Oulu, Oulu, Finland., Ebeling T; Department of Internal Medicine, Oulu University Hospital and University of Oulu, Oulu, Finland., Moilanen L; Department of Medicine, Kuopio University Hospital, Kuopio, Finland., Norvio L; Divison of Endocrinology, Helsinki University Hospital, Hyvinkää, Finland., Tamminen M; Department of Internal Medicine, Central Hospital of Kymenlaakso, Kotka, Finland., Rautiainen P; Department of Internal Medicine, Joint Muncipal Authority for North Karelia Social and Health Services (Siun sote), Joensuu, Finland., Vehkavaara S; Endocrinology, Abdominal Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland., Ryhänen E; Endocrinology, Abdominal Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland., Pekkarinen T; Endocrinology, Abdominal Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland., Matikainen N; Endocrinology, Abdominal Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland., Sane T; Endocrinology, Abdominal Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland., Schalin-Jäntti C; Endocrinology, Abdominal Center, Helsinki University Hospital and University of Helsinki, Helsinki, Finland. camilla.schalin-jantti@hus.fi.
Jazyk: angličtina
Zdroj: Endocrine [Endocrine] 2021 Nov; Vol. 74 (2), pp. 387-395. Date of Electronic Publication: 2021 May 25.
DOI: 10.1007/s12020-021-02768-0
Abstrakt: 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.
(© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
Databáze: MEDLINE
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