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Published in: World Journal of Surgery 3/2014

01-03-2014

Role of Preoperative Basal Calcitonin Levels in the Timing of Prophylactic Thyroidectomy in Patients With Germline RET Mutations

Authors: Jean-Christophe Lifante, Claire Blanchard, Eric Mirallié, Albert David, Jean-Louis Peix

Published in: World Journal of Surgery | Issue 3/2014

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Abstract

Background

The American Thyroid Association (ATA) published recommendations for the timing of prophylactic surgery for medullary thyroid carcinoma based on the specific mutation, patient age, family history, and serum calcitonin levels. The aim of this study was to assess the role of preoperative basal calcitonin (prebCt) levels in predicting the presence of medullary carcinoma of the thyroid in patients with RET mutations.

Methods

We conducted a retrospective study in two endocrine surgery departments. Between 1986 and 2012, a total of 32 patients with RET mutations underwent prophylactic thyroidectomy. The patients were stratified into four ATA risk levels: A, B, C, and D.

Results

All of the patients were biologically cured. Microcarcinoma was observed in the final pathology report for four of the 20 patients with normal prebCt (25 %) and for nine of the 12 patients with elevated prebCt (75 %). In the level A group, four patients with normal prebCt and one patient with elevated prebCt presented with microcarcinoma. In the level C group, one patient with normal prebCt and six of the seven patients with elevated prebCt (86 %) presented with microcarcinoma.

Conclusions

PrebCt can predict the presence of microcarcinoma according to surgical pathological analysis. Patients with microcarcinoma can be biochemically and clinically cured using prophylactic thyroidectomy.
Literature
1.
go back to reference Piolat C, Dyon JF, Sturm N et al (2006) Very early prophylactic thyroid surgery for infants with a mutation of the RET proto-oncogene at codon 634: evaluation of the implementation of international guidelines for MEN type 2 in a single centre. Clin Endocrinol (Oxf) 65:118–124CrossRef Piolat C, Dyon JF, Sturm N et al (2006) Very early prophylactic thyroid surgery for infants with a mutation of the RET proto-oncogene at codon 634: evaluation of the implementation of international guidelines for MEN type 2 in a single centre. Clin Endocrinol (Oxf) 65:118–124CrossRef
2.
go back to reference Skinner MA, Moley JA, Dilley WG et al (2005) Prophylactic thyroidectomy in multiple endocrine neoplasia type 2A. N Engl J Med 353:1105–1113PubMedCrossRef Skinner MA, Moley JA, Dilley WG et al (2005) Prophylactic thyroidectomy in multiple endocrine neoplasia type 2A. N Engl J Med 353:1105–1113PubMedCrossRef
3.
go back to reference Machens A, Niccoli-Sire P, Hoegel J et al (2003) Early malignant progression of hereditary medullary thyroid cancer. N Engl J Med 349:1517–1525PubMedCrossRef Machens A, Niccoli-Sire P, Hoegel J et al (2003) Early malignant progression of hereditary medullary thyroid cancer. N Engl J Med 349:1517–1525PubMedCrossRef
4.
go back to reference Wolfe HJ, Melvin KE, Cervi-Skinner SJ et al (1973) C-cell hyperplasia preceding medullary thyroid carcinoma. N Engl J Med 289:437–441PubMedCrossRef Wolfe HJ, Melvin KE, Cervi-Skinner SJ et al (1973) C-cell hyperplasia preceding medullary thyroid carcinoma. N Engl J Med 289:437–441PubMedCrossRef
5.
go back to reference Donis-Keller H, Dou S, Chi D, Carlson KM et al (1993) Mutations in the RET proto-oncogene are associated with MEN 2A and FMTC. Hum Mol Genet 2:851–856PubMedCrossRef Donis-Keller H, Dou S, Chi D, Carlson KM et al (1993) Mutations in the RET proto-oncogene are associated with MEN 2A and FMTC. Hum Mol Genet 2:851–856PubMedCrossRef
6.
go back to reference Mulligan LM, Kwok JB, Healey CS et al (1993) Germ-line mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2A. Nature 363:458–460PubMedCrossRef Mulligan LM, Kwok JB, Healey CS et al (1993) Germ-line mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2A. Nature 363:458–460PubMedCrossRef
7.
go back to reference Carlson KM, Dou S, Chi D et al (1994) Single missense mutation in the tyrosine kinase catalytic domain of the RET protooncogene is associated with multiple endocrine neoplasia type 2B. Proc Natl Acad Sci USA 91:1579–1583PubMedCrossRef Carlson KM, Dou S, Chi D et al (1994) Single missense mutation in the tyrosine kinase catalytic domain of the RET protooncogene is associated with multiple endocrine neoplasia type 2B. Proc Natl Acad Sci USA 91:1579–1583PubMedCrossRef
8.
go back to reference Hofstra RM, Landsvater RM, Ceccherini I et al (1994) A mutation in the RET proto-oncogene associated with multiple endocrine neoplasia type 2B and sporadic medullary thyroid carcinoma. Nature 367:375–376PubMedCrossRef Hofstra RM, Landsvater RM, Ceccherini I et al (1994) A mutation in the RET proto-oncogene associated with multiple endocrine neoplasia type 2B and sporadic medullary thyroid carcinoma. Nature 367:375–376PubMedCrossRef
9.
go back to reference Carlomagno F, Salvatore G, Cirafici AM et al (1997) The different RET-activating capability of mutations of cysteine 620 or cysteine 634 correlates with the multiple endocrine neoplasia type 2 disease phenotype. Cancer Res 57:391–395PubMed Carlomagno F, Salvatore G, Cirafici AM et al (1997) The different RET-activating capability of mutations of cysteine 620 or cysteine 634 correlates with the multiple endocrine neoplasia type 2 disease phenotype. Cancer Res 57:391–395PubMed
10.
go back to reference Kloos RT, Eng C, Evans DB et al (2009) Medullary thyroid cancer: management guidelines of the American Thyroid Association. Thyroid 19:565–612PubMedCrossRef Kloos RT, Eng C, Evans DB et al (2009) Medullary thyroid cancer: management guidelines of the American Thyroid Association. Thyroid 19:565–612PubMedCrossRef
11.
go back to reference Machens A, Schneyer U, Holzhausen HJ et al (2005) Prospects of remission in medullary thyroid carcinoma according to basal calcitonin level. J Clin Endocrinol Metab 90:2029–2034PubMedCrossRef Machens A, Schneyer U, Holzhausen HJ et al (2005) Prospects of remission in medullary thyroid carcinoma according to basal calcitonin level. J Clin Endocrinol Metab 90:2029–2034PubMedCrossRef
12.
go back to reference Brandi ML, Gagel RF, Angeli A (2001) Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 86:5658–5671PubMedCrossRef Brandi ML, Gagel RF, Angeli A (2001) Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 86:5658–5671PubMedCrossRef
14.
go back to reference Graham SM, Genel M, Touloukian RJ et al (1987) Provocative testing for occult medullary carcinoma of the thyroid: findings in seven children with multiple endocrine neoplasia type IIa. J Pediatr Surg 22:501–503PubMedCrossRef Graham SM, Genel M, Touloukian RJ et al (1987) Provocative testing for occult medullary carcinoma of the thyroid: findings in seven children with multiple endocrine neoplasia type IIa. J Pediatr Surg 22:501–503PubMedCrossRef
15.
go back to reference Rohmer V, Vidal-Trecan G, Bourdelot A et al (2010) Prognostic factors of disease-free survival after thyroidectomy in 170 young patients with a RET germline mutation: a multicenter study of the Groupe Francais d’Etude des Tumeurs Endocrines. J Clin Endocrinol Metab 96:E509–518PubMedCrossRef Rohmer V, Vidal-Trecan G, Bourdelot A et al (2010) Prognostic factors of disease-free survival after thyroidectomy in 170 young patients with a RET germline mutation: a multicenter study of the Groupe Francais d’Etude des Tumeurs Endocrines. J Clin Endocrinol Metab 96:E509–518PubMedCrossRef
16.
go back to reference Niccoli-Sire P, Murat A, Rohmer V et al (2003) When should thyroidectomy be performed in familial medullary thyroid carcinoma gene carriers with non-cysteine RET mutations? Surgery 134:1029–1036PubMedCrossRef Niccoli-Sire P, Murat A, Rohmer V et al (2003) When should thyroidectomy be performed in familial medullary thyroid carcinoma gene carriers with non-cysteine RET mutations? Surgery 134:1029–1036PubMedCrossRef
17.
go back to reference Niccoli-Sire P, Murat A, Rohmer V et al (2001) Familial medullary thyroid carcinoma with noncysteine ret mutations: phenotype-genotype relationship in a large series of patients. J Clin Endocrinol Metab 86:3746–3753PubMedCrossRef Niccoli-Sire P, Murat A, Rohmer V et al (2001) Familial medullary thyroid carcinoma with noncysteine ret mutations: phenotype-genotype relationship in a large series of patients. J Clin Endocrinol Metab 86:3746–3753PubMedCrossRef
18.
go back to reference Machens A, Ukkat J, Brauckhoff M et al (2005) Advances in the management of hereditary medullary thyroid cancer. J Intern Med 257:50–59PubMedCrossRef Machens A, Ukkat J, Brauckhoff M et al (2005) Advances in the management of hereditary medullary thyroid cancer. J Intern Med 257:50–59PubMedCrossRef
Metadata
Title
Role of Preoperative Basal Calcitonin Levels in the Timing of Prophylactic Thyroidectomy in Patients With Germline RET Mutations
Authors
Jean-Christophe Lifante
Claire Blanchard
Eric Mirallié
Albert David
Jean-Louis Peix
Publication date
01-03-2014
Publisher
Springer US
Published in
World Journal of Surgery / Issue 3/2014
Print ISSN: 0364-2313
Electronic ISSN: 1432-2323
DOI
https://doi.org/10.1007/s00268-013-2413-y

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