Long-term outcome after DNA-based prophylactic neck surgery in children at risk of hereditary medullary thyroid cancer

被引:18
作者
Machens, Andreas [1 ]
Dralle, Henning [1 ,2 ]
机构
[1] Martin Luther Univ Halle Wittenberg, Med Fac, Dept Visceral Vasc & Endocrine Surg, Ernst Grube Str 40, D-06097 Halle, Saale, Germany
[2] Univ Duisburg Essen, Dept Gen Visceral & Transplantat Surg, Sect Endocrine Surg, D-45122 Essen, Germany
关键词
RET proto-oncogene; DNA-based screening; medullary thyroid carcinoma; lymph node metastasis; prophylactic thyroidectomy; biochemical cure; ENDOCRINE NEOPLASIA TYPE-2; LYMPH-NODE DISSECTION; RET PROTOONCOGENE; SERUM CALCITONIN; 2A PHENOTYPE; CARCINOMA; MUTATION; PREVALENCE; CARRIERS; ORIGIN;
D O I
10.1016/j.beem.2019.04.008
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Advances in sequencing technology, providing unprecedented insights into cancer progression, have shifted the treatment paradigm towards precision medicine for hereditary medullary thyroid cancer (MTC), away from the 'one-size-fits-all' approach predicated on genetic risk alone. The DNA-based/biochemical concept, factoring serum calcitonin into the benefit-risk equation, optimizes biochemical cure while minimizing extent of prophylactic surgery and operative morbidity in children at risk. The transformative effect that has taking effect on medical practice has been impressive: Increasingly earlier molecular diagnosis and more limited prophylactic neck operations yielded excellent clinical outcomes at expert facilities 7-16 years postoperatively: biochemical cure rates approximating 100%; absence of residual structural disease or recurrence; and rarely any permanent operative morbidity. These excellent results, contingent on proper health care funding and pediatric surgical specialization, make a case for early prophylactic thyroidectomy in experienced hands once calcitonin serum levels exceed the upper normal limit of the assay in young gene carriers. (C) 2019 Elsevier Ltd. All rights reserved.
引用
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页数:18
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