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- Title
Long-Term Outcomes and Aggressiveness of Hereditary Medullary Thyroid Carcinoma: 40 Years of Experience at One Center.
- Authors
Raue, Friedhelm; Bruckner, Thomas; Frank-Raue, Karin
- Abstract
<bold>Context: </bold>Recent data on long-term outcomes and aggressiveness of medullary thyroid carcinoma (MTC) are lacking for patients with multiple endocrine neoplasia type 2 (MEN2).<bold>Objectives: </bold>To analyze the long-term outcomes in MEN2 and compare MTC aggressiveness in three defined RET mutation-risk categories: moderate risk (MOD), high risk (H), and highest risk (HST).<bold>Design, Setting: </bold>Retrospective study of 263 operated patients with MEN2 from one German tertiary referral center from 1979 to 2017 comparing demographic, biochemical, genetic, and outcome parameters.<bold>Intervention: </bold>None (observational study).<bold>Main Outcome Measure: </bold>Long-term survival and outcomes in three risk groups.<bold>Results: </bold>Surgery was performed at a mean age of 35.3 ± 18.8 (MOD, n = 122), 23.0 ± 15.7 years (H, n = 120), and 14.9 ± 9.3 (HST, n = 21) years (P < 0.05). The mean follow-up was 12.9 ± 9.8 years. Age and tumor stage at diagnosis differed among the three risk groups (P < 0.0001). Multivariate analysis of disease-specific survival (DSS) showed that increasing age [hazard ratio (HR), 1.06; 95% CI, 1.02 to 1.09], stage III/IV at diagnosis (HR, 7.39; 95% CI, 2.39 to 22.8), and HST group (HR, 14.4; 95% CI, 3.32 to 62.6) were significantly associated with worse DSS; the H group was not (P = 0.175). The DSS rates and outcomes were not different between the MOD and H groups (P = 0.179 and P = 0.893, respectively) but were significantly inferior in the HST group (P < 0.0008 and P < 0.0001, respectively).<bold>Conclusion: </bold>MTC in patients with MEN2 showed a clearly different age of onset in the different risk groups. DSS and outcomes after MTC diagnosis were similar in the MOD and H groups, suggesting similar tumor behavior. The HST group had inferior outcomes and survival vs the MOD and or H groups.
- Subjects
MEDULLARY thyroid carcinoma; AGE of onset; TUMOR diagnosis; MULTIVARIATE analysis; TUMOR classification
- Publication
Journal of Clinical Endocrinology & Metabolism, 2019, Vol 104, Issue 10, p4264
- ISSN
0021-972X
- Publication type
journal article
- DOI
10.1210/jc.2019-00516