Medullary thyroid carcinoma (MTC) occurs as both a sporadic and an inherited disease. MTC is a consistent feature of multiple endocrine neoplasia (MEN) 2A, MEN 2B, and familial non-MEN MTC (FMTC). Plasma calcitonin is a sensitive and specific marker for the presence of MTC. Genetic testing can identify mutant gene carriers, and prophylactic total thyroidectomy should be carried out in patients with the mutant gene. The outcome of MTC is progressively worse in FMTC, MEN 2A, sporadic MTC, and MEN 2B, and MEN 2B has been found to have the worst prognosis. There is a significant genotype-phenotype correlation, which allows a more sensitive individualized approach to the timing and extent of prophylactic thyroidectomy.
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Univ Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, FranceUniv Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, France
Zenaty, D.
Aigrain, Y.
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Univ Paris 07, Robert Debre Hosp, Dept Paediat Surg, F-75019 Paris, FranceUniv Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, France
Aigrain, Y.
Peuchmaur, M.
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Univ Paris 07, Robert Debre Hosp, Dept Pathol, F-75019 Paris, FranceUniv Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, France
Peuchmaur, M.
Philippe-Chomette, P.
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Univ Paris 07, Robert Debre Hosp, Dept Paediat Surg, F-75019 Paris, FranceUniv Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, France
Philippe-Chomette, P.
Baumann, C.
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Univ Paris 07, Robert Debre Hosp, Dept Med Genet, F-75019 Paris, FranceUniv Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, France
Baumann, C.
Cornelis, F.
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Hop Lariboisiere, AP HP, Med Oncogenet Dept, F-75010 Paris, FranceUniv Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, France
Cornelis, F.
Hugot, J. P.
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Univ Paris 07, Robert Debre Hosp, AP HP, Paediat Gastroenterol Dept, F-75019 Paris, FranceUniv Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, France
Hugot, J. P.
Chevenne, D.
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Univ Paris 07, Robert Debre Hosp, AP HP, Dept Biochem, F-75019 Paris, FranceUniv Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, France
Chevenne, D.
Barbu, V.
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Univ Paris 07, UPMC, St Antoine Hosp, AP HP,Biochem & Mol Biol & Genet Dept, F-75012 Paris, FranceUniv Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, France
Barbu, V.
Guillausseau, P. J.
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Univ Paris 07, Lariboisiere Hosp, AP HP, Dept Endocrinol, F-75010 Paris, FranceUniv Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, France
Guillausseau, P. J.
Schlumberger, M.
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Inst Gustave Roussy, Dept Nucl Med, F-94800 Villejuif, FranceUniv Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, France
Schlumberger, M.
Carel, J. C.
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Univ Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, FranceUniv Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, France
Carel, J. C.
Travagli, J. P.
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Inst Gustave Roussy, Dept Surg, F-94800 Villejuif, FranceUniv Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, France
Travagli, J. P.
Leger, J.
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Univ Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, FranceUniv Paris 07, Robert Debre Hosp, Ctr Reference Malad Endocriniennes Rares Croissan, Paediat Endocrinol Dept,AP HP, F-75019 Paris, France
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Department of Surgery, Univ. of California San Francisco, San Francisco, CA 94115Department of Surgery, Univ. of California San Francisco, San Francisco, CA 94115
Gertner M.E.
Kebebew E.
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Department of Surgery, Univ. of California San Francisco, San Francisco, CA 94115Department of Surgery, Univ. of California San Francisco, San Francisco, CA 94115
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Kyoto Prefectural Univ Med, Childrens Res Hosp, Div Surg, Kyoto 6020841, JapanKyoto Prefectural Univ Med, Childrens Res Hosp, Div Surg, Kyoto 6020841, Japan
Shimotake, T
Tsuda, T
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Kyoto Prefectural Univ Med, Childrens Res Hosp, Div Surg, Kyoto 6020841, JapanKyoto Prefectural Univ Med, Childrens Res Hosp, Div Surg, Kyoto 6020841, Japan
Tsuda, T
Aoi, S
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Kyoto Prefectural Univ Med, Childrens Res Hosp, Div Surg, Kyoto 6020841, JapanKyoto Prefectural Univ Med, Childrens Res Hosp, Div Surg, Kyoto 6020841, Japan
Aoi, S
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Fumino, S
Iwai, N
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Kyoto Prefectural Univ Med, Childrens Res Hosp, Div Surg, Kyoto 6020841, JapanKyoto Prefectural Univ Med, Childrens Res Hosp, Div Surg, Kyoto 6020841, Japan