Medullary thyroid carcinoma in children

被引：3
作者：
Berdelou, Amandine [1 ]
Hartl, Dana [2 ]
Al Ghuzlan, Abir [3 ]
Mirghani, Haitham [2 ]
Chougnet, Cecile [1 ]
Baudin, Eric [1 ]
Schlumberger, Martin [1 ]
Leboulleux, Sophie [1 ]
机构：
[1] Inst Gustave Roussy, Dept Med Nucl & Endocrinol Oncol, Fac Med Paris Sud, F-94805 Villejuif, France
[2] Inst Gustave Roussy, Dept Chirurg, Fac Med Paris Sud, F-94805 Villejuif, France
[3] Inst Gustave Roussy, Dept Anatomopathol, Fac Med Paris Sud, F-94805 Villejuif, France
来源：
BULLETIN DU CANCER | 2013年 / 100卷 / 7-8期
关键词：
medullary thyroid carcinoma; multiple endocrine neoplasia; hereditary disease; MULTIPLE ENDOCRINE NEOPLASIA; C-CELL HYPERPLASIA; RET PROTOONCOGENE; SERUM CALCITONIN; PRIMARY HYPERPARATHYROIDISM; PROPHYLACTIC THYROIDECTOMY; ALTERNATING COMBINATION; MALIGNANT PROGRESSION; CODON; 883; CANCER;
D O I：
10.1684/bdc.2013.1775
中图分类号：
R73 [肿瘤学];
学科分类号：
100214 ;
摘要：
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.
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收藏
页码：780 / 788
页数：9
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