Long-Term Survivorship in Multiple Endocrine Neoplasia Type 2B Diagnosed Before and in the New Millennium

The Journal of Clinical Endocrinology and Metabolism
F RaueKarin Frank-Raue

Abstract

Recent long-term outcomes and survival data are lacking for patients with multiple endocrine neoplasia type 2B (MEN2B). To analyze long-term MEN2B outcomes and define prognostic factors. 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. Surgery. Long-term survival. We identified seven familial and 68 de novo cases of MEN2B; 61 exhibited the RET M918T genotype (2 others exhibited A883F and E768D/L790T mutations). Surgery was performed at a mean age of 16.4 ± 11.2 years. The tumor stages at diagnosis for 71 patients were stage I, 15%; stage II, 6%; stage III, 35%; and stage IV, 44%. The mean follow-up was 9.6 ± 9.0 years. The outcomes were 15 (20%) cured, 9 (12%) with minimal residual disease, 19 (25%) with metastatic disease, and 10 (13%) unknown. Medullary thyroid cancer (MTC) caused 22 deaths (29%) 7.3 ± 6.2 years after diagnosis (mean age, 22.9 ± 10.7 years). The overall survival rates at 5, 10, and 20 years were 85%, 74%, and 58%, respectively. After 2000 (vs before 2000), significantly more patients had stage I and II (32% vs 11%) and more were ...Continue Reading

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Citations

Aug 9, 2018·Journal of the Endocrine Society·Friedhelm Raue, Karin Frank-Raue
Jun 1, 2019·Journal of Pediatric Endocrinology & Metabolism : JPEM·Rie MatsushitaUNKNOWN Thyroid Committee of the Japanese Society for Pediatric Endocrinology
May 31, 2019·The Journal of Clinical Endocrinology and Metabolism·Friedhelm RaueKarin Frank-Raue
Oct 8, 2018·Reviews in Endocrine & Metabolic Disorders·Sergio Pedraza-ArévaloJusto P Castaño
Jun 18, 2020·Thyroid : Official Journal of the American Thyroid Association·Annie MathewVera Tiedje
Jan 1, 2019·The Journal of Clinical Endocrinology and Metabolism·Rossella EliseiCristina Romei
Jan 5, 2021·Familial Cancer·Chakra DiwakerTushar Bandgar
Apr 5, 2021·Seminars in Cancer Biology·Jes Sloth MathiesenUlla Feldt-Rasmussen

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