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Prophylactic neck surgery for second-generation multiple endocrine neoplasia type 2B
Affiliation:1. Medical Faculty, Department of Visceral, Vascular and Endocrine Surgery, Martin Luther University Halle-Wittenberg, Ernst-Grube-Str. 40, D-06097, Halle (Saale), Germany;2. Department of General, Visceral and Transplantation Surgery, Section of Endocrine Surgery, University of Duisburg-Essen, D-45122, Essen, Germany;1. Department of General Surgery, Istituto Ospedaliero Fondazione Poliambulanza, Via Bissolati 57, Brescia, Italy;2. Hepatobiliary Surgery Unit, Foundation Policlinico Universitario A. Gemelli, Catholic University, L.go A. Gemelli, 8 Rome, Italy;1. Department of Visceral, Vascular and Endocrine Surgery, Martin-Luther-University Halle-Wittenberg, University Medical Center Halle, Halle, Germany;2. Department of General, Visceral and Transplantation Surgery, University of Heidelberg, Heidelberg, Germany;3. Department of General, Visceral, Endocrine and Transplantation Surgery, Kantonsspital St. Gallen, St. Gallen, Switzerland;4. Institute of Medical Biometry and Informatics, University of Heidelberg, Heidelberg, Germany;5. Institute for Pathology, Industriestr. 11c, 67063, Ludwigshafen, Germany;1. Department of Public Health, College of Health Science, Debre Tabor University, Debre Tabor, Ethiopia;2. Department of Preventive Medicine, School of Public Health, Addis Ababa University, Addis Ababa, Ethiopia;3. Institute of Medical Epidemiology, Biometrics, and Informatics, Martin-Luther-University Halle-Wittenberg, Germany;4. Department of Gynaecology, Martin-Luther-University Halle-Wittenberg, Germany;5. American Cancer Society, Surveillance & Health Services Research, Atlanta, GA;6. City of Hope National Medical Center, Division of Breast Surgery, Department of Surgery, Duarte, Los Angeles County, CA;1. Department for Dermatology and Venereology, Martin Luther University Halle-Wittenberg, Halle (Saale), Germany;2. Department of Dermatology and Allergy, Skin Cancer Center Hannover, Hannover Medical School, Hannover, Germany;3. Department of Dermatology, University Hospital Essen, Essen, Germany;4. Skin Cancer Center at the University Cancer Centre and National Center for Tumor Diseases Dresden, Department of Dermatology, University Hospital Carl Gustav Carus, TU Dresden, Dresden, Germany;5. SRH Wald-Klinikum Gera, Gera, Germany;6. Department of Dermatology and Allergy, University Hospital, LMU München, Munich, Germany;7. Department of Dermatology, Allergology and Phlebology, Klinikum Bremerhaven Reinkenheide GGmbH, Bremerhaven, Germany;8. Department of Dermatology, University Hospital of Regensburg, Regensburg, Germany;9. Department of Dermatology, University Hospital Augsburg, Augsburg, Germany;10. Department of Dermatology, University Hospital Schleswig-Holstein, Campus Kiel, Germany;1. Division of Surgery and Perioperative Medicine, Flinders Medical Centre, Bedford Park, Adelaide, South Australia, Australia;2. College of Medicine and Public Health, Flinders University, South Australia, Australia;3. Department of Visceral, Vascular and Endocrine Surgery, Martin Luther University Halle-Wittenberg, Halle (Saale), Saxony-Anhalt, Germany
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.
Keywords:Multiple endocrine neoplasia type 2B  Precision medicine  Medullary thyroid cancer  Prophylactic Thyroidectomy  Serum Calcitonin
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