Management of medullary thyroid carcinoma and MEN2 syndromes in childhood

Nature Reviews. Endocrinology
Steven G WaguespackGilbert J Cote

Abstract

Medullary thyroid carcinoma (MTC) and the multiple endocrine neoplasia (MEN) type 2 syndromes are rare but important endocrine diseases that are increasingly managed by pediatric providers. MTC is generally associated with a favorable prognosis when diagnosed during childhood, where it frequently occurs secondary to activating mutations in the RET proto-oncogene and arises from pre-existing C-cell hyperplasia. MEN2A accounts for 90-95% of childhood MTC cases and is most commonly due to mutations in codon 634 of RET. MEN2B is associated with the most aggressive clinical presentation of MTC and is almost always due to the Met918Thr mutation of RET. Surgery is the primary treatment and only chance of cure, although the advent of targeted therapies seems to be improving progression-free survival in advanced cases. Since the discovery of the role of RET in MEN2A, considerable advances in the management of this syndrome have occurred, and most of the children with MEN2A who have undergone early thyroidectomy will now lead full, productive lives. Strong genotype-phenotype correlations have facilitated the development of guidelines for interventions. Contemporary approaches for deciding the appropriate age at which surgery should take ...Continue Reading

References

Mar 1, 1975·Cancer·G C ChongL H Woolner
Jun 1, 1992·Archives of Internal Medicine·H F VasenC J Lips
Mar 1, 1988·Journal of Pediatric Orthopedics·C L SaltzmanN J Hopwood
Aug 30, 1973·The New England Journal of Medicine·H J WolfeA H Tashjian
Jan 1, 1966·The Journal of Pathology and Bacteriology·E D Williams, D J Pollock
Feb 1, 1983·The Journal of Pediatrics·B A Jones, J C Sisson
Dec 1, 1995·Surgery·D S O'RiordainJ A van Heerden
Feb 15, 1994·Proceedings of the National Academy of Sciences of the United States of America·K M CarlsonH Donis-Keller
Jul 1, 1993·Human Molecular Genetics·H Donis-KellerS A Wells
Jan 1, 1997·Health Psychology : Official Journal of the Division of Health Psychology, American Psychological Association·S W VernonP M Lynch
Jan 24, 1998·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·J AudrainC Lerman
May 11, 1999·JAMA : the Journal of the American Medical Association·O NilssonC Eng
Apr 6, 2000·American Journal of Medical Genetics·F J GrosfeldH F ten Kroode
Oct 20, 2000·American Journal of Medical Genetics·F J GrosfeldH F ten Kroode
Nov 10, 2000·Journal of Medical Genetics·J R Hansford, L M Mulligan
Feb 15, 2001·European Journal of Endocrinology·L NguyenUNKNOWN French Calcitonin Tumors Study Group
Mar 10, 2001·The Journal of Clinical Endocrinology and Metabolism·A MachensH Dralle
Dec 12, 2001·The Journal of Clinical Endocrinology and Metabolism·M L BrandiS J Marx
Apr 10, 2002·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·Robert F Gagel
Jun 7, 2002·Archives of Surgery·Maria A KouvarakiDouglas B Evans
Aug 10, 2002·Journal of the American College of Surgeons·González Jose M RodríguezPascual Parrilla
Apr 11, 2003·Archives of Surgery·Linwah YipDouglas B Evans
Jul 17, 2003·Clinical Endocrinology·Uberta VergaPaolo Beck-Peccoz
Aug 21, 2003·Journal of the National Cancer Institute·Jennifer BryantKatherine L Nathanson
Jan 1, 1959·The Journal of Clinical Endocrinology and Metabolism·J B HAZARDG CRILE
Oct 17, 2003·The New England Journal of Medicine·Andreas MachensUNKNOWN European Multiple Endocrine Neoplasia (EUROMEN) Study Group
Feb 27, 2004·The New England Journal of Medicine·Andreas Machens
Jul 30, 2004·Clinical & Experimental Ophthalmology·Douglas G ParkerBrett A O'Donnell
Sep 25, 2004·Clinical Chemistry·Jean-Pierre BasuyauPhilippe Brunelle
Dec 21, 2004·Journal of Internal Medicine·T Carling, R Udelsman
Dec 21, 2004·Journal of Internal Medicine·C J LipsR B Van der Luijt

❮ Previous
Next ❯

Citations

Sep 15, 2012·The American Journal of Bioethics : AJOB·Stephanie M FullertonWylie Burke
Mar 19, 2014·Best Practice & Research. Clinical Endocrinology & Metabolism·Marek Niedziela
Mar 13, 2014·Thyroid : Official Journal of the American Thyroid Association·Thereasa A RichElizabeth Grubbs
Nov 2, 2013·Journal of Pediatric Gastroenterology and Nutrition·M G SchäppiS Koletzko
Feb 24, 2016·Journal of Pediatric Surgery·John A SandovalAlpin D Malkan
Jun 4, 2014·Endocrinology and Metabolism Clinics of North America·Mimi I HuCamilo Jimenez
Jul 17, 2012·The Journal of Surgical Research·Jocelyn F BurkeHerbert Chen
Jul 15, 2015·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·Terry C LairmoreUNKNOWN AACE Endocrine Surgery Scientific Committee
Mar 27, 2015·Thyroid : Official Journal of the American Thyroid Association·Samuel A WellsUNKNOWN American Thyroid Association Guidelines Task Force on Medullary Thyroid Carcinoma
Mar 10, 2017·Thyroid : Official Journal of the American Thyroid Association·Karine C RodriguesDelmar M Lourenço
Mar 4, 2015·Otolaryngology--head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery·Thomas E HeinemanDavid I Kutler
Jun 27, 2017·Endocrine-related Cancer·Frederic CastinettiHartmut P H Neumann
Jul 13, 2017·Endocrine-related Cancer·Frederic CastinettiSteven G Waguespack
Jan 21, 2014·Nature Reviews. Cancer·Patricia L M Dahia
Mar 21, 2013·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·Marcio L GriebelerGeoffrey B Thompson
Nov 7, 2012·Current Opinion in Endocrinology, Diabetes, and Obesity
Apr 3, 2020·Annals of the Royal College of Surgeons of England·O HamdyI H Metwally
Jun 13, 2019·Aging·Frederic Castinetti
Feb 22, 2012·Current Opinion in Oncology·Madson Q Almeida, Ana O Hoff
Jan 1, 2014·International Journal of Endocrine Oncology·Maria E CabanillasGilbert J Cote
Sep 22, 2019·Genes·Vera A PaulsonDouglas S Hawkins
Jan 1, 2016·Expert Opinion on Orphan Drugs·Diana BradfordShivaani Kummar
Sep 30, 2015·The Journal of Clinical Endocrinology and Metabolism·Keith C BibleUNKNOWN International Thyroid Oncology Group Correlative Science Committee
Jan 1, 2019·The Journal of Clinical Endocrinology and Metabolism·Rossella EliseiCristina Romei
Mar 15, 2013·Current Opinion in Otolaryngology & Head and Neck Surgery·Vincent Vander PoortenPierre Delaere
Mar 27, 2018·Journal of Ultrasound in Medicine : Official Journal of the American Institute of Ultrasound in Medicine·Sarah OgleMira Milas
Aug 9, 2020·Scientific Reports·Zhuang ZhaoDun-Wei Wang
Jun 15, 2013·Clinical Cancer Research : an Official Journal of the American Association for Cancer Research·Elizabeth FoxFrank M Balis
Jul 9, 2013·Bulletin du cancer·Amandine BerdelouSophie Leboulleux
Nov 7, 2020·Endocrinology and Metabolism Clinics of North America·Andrew J Bauer
Apr 12, 2021·Surgery·Jamie R OliverBabak Givi

❮ Previous
Next ❯

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