Prophylactic neck surgery for second-generation multiple endocrine neoplasia type 2B.

European Journal of Surgical Oncology : the Journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
Andreas MachensHenning Dralle

Abstract

There is no evidence-based guidance on the extent of prophylactic neck surgery for second-generation multiple endocrine neoplasia type 2B (MEN 2B), a newly emerging entity in the molecular era. In this investigation of MEN 2B children who inherited the M918T RET germline mutation from a phenotypically affected MEN 2B parent, 6 MEN 2B children (4 girls and 2 boys) from 5 MEN 2B parents (4 mothers and 1 father) were identified. None of the 6 second-generation MEN 2B children who had preoperative calcitonin serum levels between 2 and 105 pg/mL and underwent prophylactic total thyroidectomy before the age of 4 years after receiving a positive RET gene test harbored node metastases. There was no recurrent laryngeal nerve palsy or postoperative hypoparathyroidism. Within the limitations of this study, total thyroidectomy alone is adequate therapy for second-generation MEN 2B children aged 1-4 years old with preoperative calcitonin serum levels ≤100 pg/mL.

References

Dec 6, 2012·European Journal of Endocrinology·Andreas MachensHenning Dralle
Mar 27, 2015·Thyroid : Official Journal of the American Thyroid Association·Samuel A WellsUNKNOWN American Thyroid Association Guidelines Task Force on Medullary Thyroid Carcinoma
May 9, 2015·The Journal of Clinical Endocrinology and Metabolism·Maria G CastagnaFurio Pacini
Apr 26, 2017·Endocrine-related Cancer·Jes Sloth MathiesenChristian Godballe
Jan 18, 2018·The British Journal of Surgery·A MachensH Dralle
Mar 14, 2018·The British Journal of Surgery·R SchneiderH Dralle
Aug 17, 2018·The Journal of Clinical Endocrinology and Metabolism·Angeliki MakriMaya Lodish
Jan 1, 2019·The Journal of Clinical Endocrinology and Metabolism·Rossella EliseiCristina Romei

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