A1 Vertaisarvioitu alkuperäisartikkeli tieteellisessä lehdessä
Characteristics and outcomes of the Finnish ectopic ACTH syndrome cohort
Tekijät: 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
Kustantaja: SPRINGER
Julkaisuvuosi: 2021
Journal: Endocrine
Tietokannassa oleva lehden nimi: ENDOCRINE
Lehden akronyymi: ENDOCRINE
Vuosikerta: 74
Numero: 2
Aloitussivu: 387
Lopetussivu: 395
Sivujen määrä: 9
ISSN: 1355-008X
eISSN: 1559-0100
DOI: https://doi.org/10.1007/s12020-021-02768-0
Verkko-osoite: https://doi.org/10.1007/s12020-021-02768-0
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.
