Department of Surgery, Maine Medical Center, Portland, Maine, USA.
Curr Opin Oncol. 2011 Jan;23(1):22-7. doi: 10.1097/CCO.0b013e328340b527.
Medullary thyroid cancer (MTC) is an uncommon malignancy. Its low incidence has limited both widespread clinical expertise and definitive large randomized clinical trials. Variation in practice patterns exist in the United States with regard to diagnosis, treatment, and long-term management. We review the most recent guidelines on management of this challenging neuroendocrine malignancy.
Newly identified re-arranged during transfection point mutations have added to clinicians' disease prognostic accuracy, which have been incorporated in the new MTC treatment guidelines. The study of tumor marker doubling times has guided the extent of surgery and lymphadenectomy for MTC. Although data are limited, standard chemotherapy and radiation therapy have not been shown to be effective in the treatment of MTC. Newer targeted drug therapies are promising and are being examined in therapeutic clinical trials.
There have been several recent advances in the molecular biology, diagnosis, imaging, and treatment options for MTC. Downstaging and treating metastatic disease more effectively may improve overall survival of MTC patients. Dissemination of standardized guidelines is important for optimal treatment with less variation in quality of care.
甲状腺髓样癌（MTC）是一种罕见的恶性肿瘤。其发病率低，限制了广泛的临床专业知识和明确的大型随机临床试验。美国在诊断、治疗和长期管理方面存在实践模式的差异。我们回顾了关于这种具有挑战性的神经内分泌恶性肿瘤管理的最新指南。
新发现的重排基因在转录点突变增加了临床医生对疾病预后的准确性，这已被纳入新的 MTC 治疗指南。肿瘤标志物倍增时间的研究指导了 MTC 的手术和淋巴结清扫范围。尽管数据有限，但标准的化疗和放疗在治疗 MTC 方面并未显示出有效性。较新的靶向药物治疗有希望，并正在治疗临床试验中进行检查。
MTC 的分子生物学、诊断、影像学和治疗选择方面取得了多项最新进展。降期和更有效地治疗转移性疾病可能会改善 MTC 患者的总体生存率。传播标准化指南对于获得最佳治疗效果和减少护理质量差异非常重要。
