Management of medullary thyroid carcinoma

被引:62
作者
Jimenez, Camilo [1 ]
Hu, Mim I-Nan [1 ]
Gagel, Robert F. [1 ]
机构
[1] Univ Texas MD Anderson Canc Ctr, Dept Endocrine Neoplasia & Hormonal Disorders, Div Internal Med, Houston, TX 77030 USA
关键词
D O I
10.1016/j.ecl.2008.03.001
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Medullary thyroid carcinoma (MTC) is responsible for 13.4% of the total deaths attributable to thyroid cancer in human beings and research on MTC over the last 40 years has identified the RET proto-oncogene as a very relevant component of development of both sporadic and hereditary MTC. An activating germline RET proto-oncogene mutation responsible for a multiple endocrine neoplasia syndrome type 2 (MEN2) or a familial hereditary MTC syndrome is carried by 25% to 35% of patients with MTC. The recognition of RET proto-oncogene mutations by genetic sequencing has allowed us to differentiate hereditary from sporadic MTC, so that it is now possible to identify and treat children at risk for this disease before development of metastasis. Thanks to this discovery, we can now establish the association of MTC with other tumors in the context of MEN2 syndrome; determine adequate follow-up, prognosis, and treatment for patients with hereditary disease; and use this information to develop new therapies against both sporadic and hereditary MTCs.
引用
收藏
页码:481 / +
页数:18
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