Surgical approach to medullary thyroid carcinoma associated with multiple endocrine neoplasia type 2

dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorTAVARES, Marcos R.
dc.contributor.authorTOLEDO, Sergio P. A.
dc.contributor.authorMONTENEGRO, Fabio L. M.
dc.contributor.authorMOYSES, Raquel A.
dc.contributor.authorTOLEDO, Rodrigo A.
dc.contributor.authorSEKYIA, Tomoko
dc.contributor.authorCERNEA, Claudio R.
dc.contributor.authorBRANDAO, Lenine G.
dc.date.accessioned2013-07-30T14:41:48Z
dc.date.available2013-07-30T14:41:48Z
dc.date.issued2012
dc.description.abstractWe briefly review the surgical approaches to medullary thyroid carcinoma associated with multiple endocrine neoplasia type 2 (medullary thyroid carcinoma/multiple endocrine neoplasia type 2). The recommended surgical approaches are usually based on the age of the affected carrier/patient, tumor staging and the specific rearranged during transfection codon mutation. We have focused mainly on young children with no apparent disease who are carrying a germline rearranged during transfection mutation. Successful management of medullary thyroid carcinoma in these cases depends on early diagnosis and treatment. Total thyroidectomy should be performed before 6 months of age in infants carrying the rearranged during transfection 918 codon mutation, by the age of 3 years in rearranged during transfection 634 mutation carriers, at 5 years of age in carriers with level 3 risk rearranged during transfection mutations, and by the age of 10 years in level 4 risk rearranged during transfection mutations. Patients with thyroid tumor >5 mm detected by ultrasound, and basal calcitonin levels >40 pg/ml, frequently have cervical and upper mediastinal lymph node metastasis. In the latter patients, total thyroidectomy should be complemented by extensive lymph node dissection. Also, we briefly review our data from a large familial medullary thyroid carcinoma genealogy harboring a germline rearranged during transfection Cys620Arg mutation. All 14 screened carriers of the rearranged during transfection Cys620Arg mutation who underwent total thyroidectomy before the age of 12 years presented persistently undetectable serum levels of calcitonin (<2 pg/ml) during the follow-up period of 2-6 years. Although it is recommended that preventive total thyroidectomy in rearranged during transfection codon 620 mutation carriers is performed before the age of 5 years, in this particular family the surgical intervention performed before the age of 12 years led to an apparent biochemical cure.
dc.description.indexMEDLINE
dc.description.sponsorshipCNPq [401990/2010-9]
dc.description.sponsorshipFAPESP [11942/2009, 2009/15386-6]
dc.identifier.citationCLINICS, v.67, suppl.1, p.149-154, 2012
dc.identifier.doi10.6061/clinics/2012(Sup01)25
dc.identifier.issn1807-5932
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/452
dc.language.isoeng
dc.publisherHOSPITAL CLINICAS, UNIV SAO PAULO
dc.relation.ispartofClinics
dc.rightsopenAccess
dc.rights.holderCopyright HOSPITAL CLINICAS, UNIV SAO PAULO
dc.subjectCarcinoma
dc.subjectMedullary
dc.subjectMultiple Endocrine Neoplasia
dc.subjectSurgery
dc.subject.otherret protooncogene
dc.subject.otherprophylactic thyroidectomy
dc.subject.othercancer
dc.subject.otherpheochromocytoma
dc.subject.otherguidelines
dc.subject.otherexperience
dc.subject.otherdiagnosis
dc.subject.otherdissection
dc.subject.othermutations
dc.subject.othercarriers
dc.subject.wosMedicine, General & Internal
dc.titleSurgical approach to medullary thyroid carcinoma associated with multiple endocrine neoplasia type 2
dc.typearticle
dc.type.categoryreview
dc.type.versionpublishedVersion
dspace.entity.typePublication
hcfmusp.citation.scopus8
hcfmusp.contributor.author-fmusphcMARCOS ROBERTO TAVARES
hcfmusp.contributor.author-fmusphcSERGIO PEREIRA DE ALMEIDA TOLEDO
hcfmusp.contributor.author-fmusphcFABIO LUIZ DE MENEZES MONTENEGRO
hcfmusp.contributor.author-fmusphcRAQUEL AJUB MOYSES
hcfmusp.contributor.author-fmusphcRODRIGO DE ALMEIDA TOLEDO
hcfmusp.contributor.author-fmusphcTOMOKO SEKIYA
hcfmusp.contributor.author-fmusphcCLAUDIO ROBERTO CERNEA
hcfmusp.contributor.author-fmusphcLENINE GARCIA BRANDAO
hcfmusp.description.beginpage149
hcfmusp.description.endpage154
hcfmusp.description.issuesuppl 1
hcfmusp.description.volume67
hcfmusp.lim.ref2012
hcfmusp.origemWOS
hcfmusp.origem.pubmed22584721
hcfmusp.origem.scieloSCIELO:S1807-59322012001300025
hcfmusp.origem.scopus2-s2.0-84863914510
hcfmusp.origem.wosWOS:000304082400025
hcfmusp.publisher.citySAO PAULO
hcfmusp.publisher.countryBRAZIL
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hcfmusp.remissive.sponsorshipCNPq
hcfmusp.remissive.sponsorshipFAPESP
hcfmusp.scopus.lastupdate2024-06-16
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