Characteristics and outcomes of the Finnish ectopic ACTH syndrome cohort
| Autor: | Leena Norvio, Timo Sane, Päivi O. Hämäläinen, Helena Leijon, Eeva Ryhänen, Minna Soinio, Camilla Schalin-Jäntti, Päivi Rautiainen, Tapani Ebeling, Leena Moilanen, Otto Knutar, Minna Koivikko, Sanna Toivanen, Saara Metso, Aura Arola, Tuula Pekkarinen, Niina Matikainen, Satu Vehkavaara, Marjo Tamminen |
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| Rok vydání: | 2021 |
| Předmět: |
medicine.medical_specialty
Delayed Diagnosis Endocrinology Diabetes and Metabolism 030209 endocrinology & metabolism 03 medical and health sciences 0302 clinical medicine Endocrinology Internal medicine Diabetes mellitus medicine Humans Neuroendocrine carcinoma Finland Metyrapone business.industry Incidence (epidemiology) medicine.disease Primary tumor Occult ACTH Syndrome Ectopic Neuroendocrine Tumors 030220 oncology & carcinogenesis Ectopic ACTH syndrome Cohort Neoplasm Recurrence Local business medicine.drug |
| Zdroj: | Endocrine. 74:387-395 |
| ISSN: | 1559-0100 1355-008X 1997-2016 |
| Popis: | Ectopic ACTH syndrome (EAS) is rare. We established a national cohort to increase awareness and address unmet needs.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).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.NET/NEC may precede EAS onset by several years. In NETG1/G2, recurrences may occur10 years after successful primary surgery. Tumor subgroup (OT, NETG1/G2, NEC) was an independent predictor of survival. |
| Databáze: | OpenAIRE |
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