Tiroidectomía profiláctica en niños y jóvenes con cáncer medular tiroideo hereditario: Experiencia chilena

Background: With the availability of the RET proto-oncogene genetic testing, it is possible to perform prophylactic total thyroidectomy among carriers of RET mutation. Aim: To evaluate the histological findings and the effects of the prophylactic total thyroidectomy in first-degree relatives of Chil...

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Autores principales:	Ramos S,Claudia, Ojeda S,Dagoberto, Cohn M,David, Wohllk G,Nelson
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2005
Materias:	Multiple endocrine neoplasia type 2 Thyroidectomy Thyroid neoplasms)
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872005000900005
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spelling	oai:scielo:S0034-988720050009000052005-11-09Tiroidectomía profiláctica en niños y jóvenes con cáncer medular tiroideo hereditario: Experiencia chilenaRamos S,ClaudiaOjeda S,DagobertoCohn M,DavidWohllk G,Nelson Multiple endocrine neoplasia type 2 Thyroidectomy Thyroid neoplasms) Background: With the availability of the RET proto-oncogene genetic testing, it is possible to perform prophylactic total thyroidectomy among carriers of RET mutation. Aim: To evaluate the histological findings and the effects of the prophylactic total thyroidectomy in first-degree relatives of Chilean patients with multiple endocrine neoplasia type 2 (MEN 2) based on the Ret proto-oncogen analysis. Subjects and Methods: Nineteen patients belonging to 11 MEN 2 families underwent total thyroidectomy. Of these, 16 either with C cell hyperplasia (CCH) or microscopic medullary thyroid carcinoma (MTC) were selected for the final analysis. Results: The age at the moment of thyroidectomy ranged from 3 to 24 years (median 9.5). The most common mutation was located in codon 634 (69%) followed by codon 620 (25%). Histopathology revealed MTC in 13 patients (81%, youngest 3 years, oldest ones 19 and 24 years) and CCH in 3. A significant correlation was observed between basal preoperative serum calcitonin/tumor size (r= 0.53, P <0.05) and age/tumor size (r= 0.56, P <0.03), but not between basal preoperative serum calcitonin and age. Stimulated preoperative calcitonin levels were confounding and not useful for differentiating CCH from MTC. None of patients in whom cervical dissection was done (9/16) presented lymph node metastases, including the oldest ones. All patients but the older ones were biochemically cured after a mean of 5 years of follow-up. Conclusion: Prophylactic total thyroidectomy should be done early in life because there is an age-dependent progression from HCC to MTC. MTC often precedes biochemical detection of the disease (Rev Méd Chile 2005; 133: 1029-36)info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.133 n.9 20052005-09-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872005000900005es10.4067/S0034-98872005000900005
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Multiple endocrine neoplasia type 2 Thyroidectomy Thyroid neoplasms)
spellingShingle	Multiple endocrine neoplasia type 2 Thyroidectomy Thyroid neoplasms) Ramos S,Claudia Ojeda S,Dagoberto Cohn M,David Wohllk G,Nelson Tiroidectomía profiláctica en niños y jóvenes con cáncer medular tiroideo hereditario: Experiencia chilena
description	Background: With the availability of the RET proto-oncogene genetic testing, it is possible to perform prophylactic total thyroidectomy among carriers of RET mutation. Aim: To evaluate the histological findings and the effects of the prophylactic total thyroidectomy in first-degree relatives of Chilean patients with multiple endocrine neoplasia type 2 (MEN 2) based on the Ret proto-oncogen analysis. Subjects and Methods: Nineteen patients belonging to 11 MEN 2 families underwent total thyroidectomy. Of these, 16 either with C cell hyperplasia (CCH) or microscopic medullary thyroid carcinoma (MTC) were selected for the final analysis. Results: The age at the moment of thyroidectomy ranged from 3 to 24 years (median 9.5). The most common mutation was located in codon 634 (69%) followed by codon 620 (25%). Histopathology revealed MTC in 13 patients (81%, youngest 3 years, oldest ones 19 and 24 years) and CCH in 3. A significant correlation was observed between basal preoperative serum calcitonin/tumor size (r= 0.53, P <0.05) and age/tumor size (r= 0.56, P <0.03), but not between basal preoperative serum calcitonin and age. Stimulated preoperative calcitonin levels were confounding and not useful for differentiating CCH from MTC. None of patients in whom cervical dissection was done (9/16) presented lymph node metastases, including the oldest ones. All patients but the older ones were biochemically cured after a mean of 5 years of follow-up. Conclusion: Prophylactic total thyroidectomy should be done early in life because there is an age-dependent progression from HCC to MTC. MTC often precedes biochemical detection of the disease (Rev Méd Chile 2005; 133: 1029-36)
author	Ramos S,Claudia Ojeda S,Dagoberto Cohn M,David Wohllk G,Nelson
author_facet	Ramos S,Claudia Ojeda S,Dagoberto Cohn M,David Wohllk G,Nelson
author_sort	Ramos S,Claudia
title	Tiroidectomía profiláctica en niños y jóvenes con cáncer medular tiroideo hereditario: Experiencia chilena
title_short	Tiroidectomía profiláctica en niños y jóvenes con cáncer medular tiroideo hereditario: Experiencia chilena
title_full	Tiroidectomía profiláctica en niños y jóvenes con cáncer medular tiroideo hereditario: Experiencia chilena
title_fullStr	Tiroidectomía profiláctica en niños y jóvenes con cáncer medular tiroideo hereditario: Experiencia chilena
title_full_unstemmed	Tiroidectomía profiláctica en niños y jóvenes con cáncer medular tiroideo hereditario: Experiencia chilena
title_sort	tiroidectomía profiláctica en niños y jóvenes con cáncer medular tiroideo hereditario: experiencia chilena
publisher	Sociedad Médica de Santiago
publishDate	2005
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872005000900005
work_keys_str_mv	AT ramossclaudia tiroidectomiaprofilacticaenninosyjovenesconcancermedulartiroideohereditarioexperienciachilena AT ojedasdagoberto tiroidectomiaprofilacticaenninosyjovenesconcancermedulartiroideohereditarioexperienciachilena AT cohnmdavid tiroidectomiaprofilacticaenninosyjovenesconcancermedulartiroideohereditarioexperienciachilena AT wohllkgnelson tiroidectomiaprofilacticaenninosyjovenesconcancermedulartiroideohereditarioexperienciachilena
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Tiroidectomía profiláctica en niños y jóvenes con cáncer medular tiroideo hereditario: Experiencia chilena

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