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儿童和青少年甲状腺癌

Thyroid cancer in children and adolescents.

作者信息

Vander Poorten Vincent, Hens Greet, Delaere Pierre

机构信息

Department of Otorhinolaryngology, Head and Neck Surgery, Leuven Cancer Institute, University Hospitals Leuven, Leuven, Belgium.

出版信息

Curr Opin Otolaryngol Head Neck Surg. 2013 Apr;21(2):135-42. doi: 10.1097/MOO.0b013e32835e15d9.

Abstract

PURPOSE OF REVIEW

Paediatric thyroid cancer is a rare disease, but its incidence is rising in recent reports. This review aims at integrating recent findings into the current optimal diagnostic and therapeutic approach.

RECENT FINDINGS

The causal relationship of differentiated thyroid cancer (DTC) to radiation exposure is increasingly unravelled. Research progressively uncovers the genetic basis, such as RET (rearranged during transfection)/papillary thyroid cancer (PTC) rearrangement and RET-mutations. Knowledge of oncogenic signalling pathways nowadays starts to help finetuning diagnosis, prognosis and treatment. This knowledge complements the current state-of-the-art of paediatric thyroid cancer treatment. In childhood, DTC presents at a more advanced stage and implies higher recurrence rates, recurrences often occurring decades later. Treatment should minimize not only these recurrences but also long-term treatment sequelae. Total thyroidectomy and central compartment dissection by a high-volume surgeon and radioactive iodine is the preferred approach for most children with DTC. For children with medullary thyroid cancer within the MEN2 framework, when possible, prophylactic thyroidectomy is performed. Unfortunately, frequently, the diagnosis is still made at a later stage, and then requires total thyroidectomy with dissection of the central compartment and the lateral neck, when involved.

SUMMARY

The management complexity, the essential long-term follow-up and the lifetime burden of eventual complications demands management of paediatric thyroid cancer by physicians with the highest expertise. In such hands, excellent results can be obtained.

摘要

综述目的

儿童甲状腺癌是一种罕见疾病,但近期报告显示其发病率呈上升趋势。本综述旨在将近期研究结果整合到当前最佳诊断和治疗方法中。

近期研究结果

分化型甲状腺癌(DTC)与辐射暴露之间的因果关系越来越清晰。研究逐渐揭示了其遗传基础,如RET(转染重排)/甲状腺乳头状癌(PTC)重排和RET突变。目前,对致癌信号通路的了解开始有助于优化诊断、预后和治疗。这些知识补充了当前儿童甲状腺癌治疗的先进水平。在儿童时期,DTC多在较晚期出现,意味着复发率较高,复发常发生在数十年后。治疗不仅应尽量减少这些复发,还应尽量减少长期治疗后遗症。对于大多数DTC患儿,由经验丰富的外科医生进行全甲状腺切除术和中央区淋巴结清扫,并给予放射性碘治疗是首选方法。对于MEN2综合征框架内的髓样甲状腺癌患儿,尽可能进行预防性甲状腺切除术。不幸的是,诊断往往仍在较晚期做出,此时需要进行全甲状腺切除术,并清扫中央区淋巴结,如有必要还需清扫侧颈部淋巴结。

总结

管理的复杂性、必要的长期随访以及最终并发症的终身负担要求由最专业的医生来管理儿童甲状腺癌。在这些医生的手中,可以取得优异的治疗效果。

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