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Bull Cancer. 2013 Jul-Aug;100(7-8):780-8. doi: 10.1684/bdc.2013.1775.

[Medullary thyroid carcinoma in children].

[Article in French]

Author information

1
Institut Gustave-Roussy, Département de Médecine Nucléaire et d'Endocrinologie Oncologique, Faculté de Médecine Paris-Sud, 39 rue Camille-Desmoulins, 94805 Villejuif cedex, France.

Abstract

Medullary thyroid carcinoma (MTC) is rare in children. MTC is almost always inherited and occurs as part of a multiple endocrine neoplasia type 2A and B, due to germline mutation in the RET proto-oncogene. MTC in the pediatric population is most often diagnosed in the course of a familial genetic investigation. But when the child is the proband, a de novo mutation is most often founded. The main aim is to treat MTC before extrathyroidal extension occurs because when distant metastases are present, it is rarely curable. Treatment is based on total thyroidectomy with cervical lymph node dissection.

KEYWORDS:

hereditary disease; medullary thyroid carcinoma; multiple endocrine neoplasia

PMID:
23831771
DOI:
10.1684/bdc.2013.1775
[Indexed for MEDLINE]
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