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Chirurgisches Vorgehen bei Kindern mit medullärem Schilddrüsenkarzinom unter Berücksichtigung der multiplen endokrinen Neoplasie Typ II

  • K. Schäfer
  • N. Senninger
  • H. Roth
  • H. J. Buhr
Conference paper
Part of the Langenbecks Archiv für Chirurgie book series (DTGESCHIR, volume 1996)

Operative Strategy in Childen and Adolescents with Medullary Carcinoma of the Thyroid (MTC) Including Multiple Endocrine Neoplasia (MEN) Type II

Summary

Between 1988 and 1995 we treated ten patients aged 7–18 years for familial MTC or MEN II syndrome with a total of 17 operations. Using these surgical procedures, 6/9 patients remained free of tumor after 0–8 years (mean 4.5 years).

In conclusion, we recommend thyroidectomy [1] in children younger than 5 years of age and a family history of MEN IIa; [2] in children younger than 3 years of age, presenting the characteristic phenotype of MEN IIb, both with a positive test for mutation of the RET protooncogene [4].

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Literatur

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    Buhr HJ, Raue F, Herfarth C (1991) Spezielle Tumorbiologie und Chirurgie des C-Zell Karzinoms. Chirurg 62: 529–535PubMedGoogle Scholar
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    Decker RA, Peacock ML, Borst MJ, Sweet JD, Thompson NW (1995) Progress in genetic screening of multiple endocrine neoplasia type 2a: is calcitonin testing obsolete? Surgery 118: 257–263PubMedCrossRefGoogle Scholar
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    Dralle H, Hüppner W, Raue F (1996) Prophylaktische Thyreoidektomie. Dt Ärztebl 93: 899–901Google Scholar
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    Dralle H, Scheumann GF, Kotzerke J, Brabant EG (1992) Surgical management of MEN 2. Recent Results Cancer Res 125: 167–195PubMedCrossRefGoogle Scholar
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    Frilling A, Hoppner W, Eng C, Mulligan L, Raue F, Broelsch CE (1995) Presymptomatic genetic screening in families with multiple endocrine neoplasia type 2. J Mol Med 73: 229–233PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1996

Authors and Affiliations

  • K. Schäfer
    • 1
  • N. Senninger
    • 1
  • H. Roth
    • 1
  • H. J. Buhr
    • 1
  1. 1.Abteilung Kinderchirurgie, Chirurgische KlinikRuprecht-Karls-UniversitätHeidelbergDeutschland

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