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J Clin Endocrinol Metab. 2019 May 30. pii: jc.2019-00516. doi: 10.1210/jc.2019-00516. [Epub ahead of print]

Long-Term Outcomes and Aggressiveness of Hereditary Medullary Thyroid Carcinoma: 40 Years of Experience at One Center.

Author information

1
Endocrine Practice Heidelberg, Molecular Genetic Laboratory, Heidelberg, Germany.
2
Institute of Medical Biometry and Informatics, University of Heidelberg, Heidelberg, Germany.

Abstract

CONTEXT:

Recent data on long-term outcomes and aggressiveness of medullary thyroid carcinoma (MTC) are lacking for patients with multiple endocrine neoplasia type 2 (MEN2).

OBJECTIVES:

To analyze long-term outcome in MEN2 and compare MTC aggressiveness in 3 defined RET mutation risk categories: moderate risk (MOD), high risk (H), and highest risk (HST).

DESIGN, SETTING:

Retrospective study of 263 operated patients with MEN2 from one German tertiary referral center between 1979 and 2017, comparing demographic, biochemical, genetic, and outcome parameters.

INTERVENTION:

None (observational study).

MAIN OUTCOME MEASURE:

Long-term survival and outcomes in 3 risk groups.

RESULTS:

Surgery was performed at mean ages of 35.3±18.8 (MOD, n=122) 23.0±15.7 years (H, n=120), and 14.9±9.3 years (HST, n=21) (p< 0.05) in the different risk categories. Mean follow-up was 12.9±9.8 years. Age and tumor stage at diagnosis differed among the 3 risk groups (p<0.0001). In a multivariate analysis for disease-specific survival (DSS), increasing age [HR=1.06 (95%CI=1.02-1.09)], stage III/IV at diagnosis [HR=7.39 (2.39-22.8)], and HST risk group [HR=14.4 (3.32-62.6)] were significantly associated with worse DSS; H group membership was not (P=0.175). DSS rates and outcome were not different between MOD and H groups (p=0.179, p=0.893), but were significantly inferior in the HST group (p<0.0008, P<0.0001).

CONCLUSION:

MTC in MEN2 patients has a clearly different age of onset in different risk groups. DSS and outcome after MTC diagnosis are similar in MOD and H groups suggesting similar tumor behavior. HST patients had significantly inferior outcomes and survival versus MOD or H groups.

PMID:
31145454
DOI:
10.1210/jc.2019-00516

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