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Long-Term Survivorship in Multiple Endocrine Neoplasia Type 2B diagnosed before and in the new Millennium.

Long-Term Survivorship in Multiple Endocrine Neoplasia Type 2B diagnosed before and in the new Millennium.
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Raue F, Dralle H, Machens A, Bruckner T, Frank-Raue K,


Raue F, Dralle H, Machens A, Bruckner T, Frank-Raue K, (click to view)

Raue F, Dralle H, Machens A, Bruckner T, Frank-Raue K,

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The Journal of clinical endocrinology and metabolism 2017 10 25() doi 10.1210/jc.2017-01884

Abstract
Context
Recent long-term outcome and survival data are lacking for patients with multiple endocrine neoplasia type 2B (MEN2B).

Objectives
To analyze long-term MEN2B outcome and define prognostic factors.

Design, Setting, and Participants
Retrospective comparative study of 75 patients with MEN2B from two German tertiary referral centers. Patients diagnosed and treated before and after 2000 were compared for demographic, biochemical, surgical, and outcome parameters.

Intervention
Surgery.

Main Outcome measure
Long-term survival.

Results
We identified 7 familial and 68 de novo cases of MEN2B; 61 cases exhibited the RET M918T genotype (two others exhibited A883F and E768D/L790T mutations). Surgery was performed at a mean age of 16.4±11.2 years. Tumor stages at diagnosis were (n=71 patients): stage I: 15%; stage II: 6%; stage III: 35%; stage IV: 44%. The mean follow up was 9.6±9.0 years. Outcomes were: n=15 (20%) cured, n= 9 (12%) minimal residual disease, n=19 (25%) metastatic disease, and n=10 (13%) no information. Medullary thyroid cancer (MTC) caused 22 (29%) deaths at 7.3±6.2 years after diagnosis (mean age 22.9±10.7 years). Overall survival rates at 5, 10, and 20 years were 85%, 74%, and 58%, respectively. After 2000 (vs. before 2000), significantly more patients were in stages I and II (32% vs. 11%), more were cured (43% vs. 20%), and we observed a higher survival trend (p=0.058). The only prognostic factor was tumor stage at diagnosis.

Conclusion
Patients with MEN2B developed MTC at an early age with wide ranging aggressiveness, but the outcome was generally better after 2000 than before 2000.

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