Diesen Diana L, Skinner Michael A
Children's Medical Center Dallas, UT Southwestern Medical Center, Dallas, Texas, USA.
Semin Pediatr Surg. 2012 Feb;21(1):44-50. doi: 10.1053/j.sempedsurg.2011.10.004.
Thyroid cancer is an uncommon childhood malignancy that presents primarily in young children or adolescent females and may be related to radiation exposure or genetic predisposition. Gene alterations, such as RET mutation or RET/PTC rearrangement, are not uncommon. Recent studies have lead to an increased understanding of the role of these particular gene alterations in the diagnosis, prognosis, and treatment of thyroid cancer. Surgery remains the mainstay of treatment for thyroid cancer followed by radioactive iodine when appropriate. In patients with MEN2, prophylactic thyroidectomy is recommended, although a delay in the initial diagnosis is common. With early aggressive treatment and long-term follow-up, these patients generally have excellent outcomes. Recent research suggests potential usefulness of novel therapies directed at oncogenic signaling pathways, modulators of growth, angiogenesis inhibitors, immunomodulators, and gene therapy.
甲状腺癌是一种罕见的儿童恶性肿瘤,主要发生于幼儿或青春期女性,可能与辐射暴露或遗传易感性有关。基因改变,如RET突变或RET/PTC重排并不罕见。最近的研究增进了人们对这些特定基因改变在甲状腺癌诊断、预后和治疗中作用的理解。手术仍然是甲状腺癌治疗的主要手段,必要时辅以放射性碘治疗。对于患有MEN2的患者,建议进行预防性甲状腺切除术,不过初始诊断往往会延迟。通过早期积极治疗和长期随访,这些患者通常有良好的预后。最近的研究表明,针对致癌信号通路、生长调节剂、血管生成抑制剂、免疫调节剂和基因治疗的新型疗法可能具有实用性。