摘要
少见类型甲状腺恶性肿瘤（包括甲状腺未分化癌、甲状腺髓样癌、原发性甲状腺淋巴瘤、甲状腺鳞状细胞癌、甲状腺低分化癌及甲状腺肉瘤）虽占所有甲状腺癌不足10%，却导致＞50%的甲状腺癌相关死亡，构成严峻的临床挑战。此类肿瘤具有侵袭性强、诊断困难、初诊转移发生率高、治疗手段有限及预后不良等特点。当前诊疗困境主要在于诊断延误、分期不精准以及治疗方案缺乏统一规范。尽管近年来随着靶向药物研发的推进及外科手术技术与理念的革新，部分病人的预后有所改善，但总体生存率的提升仍不显著。针对该类病人，应建立以外科为主导的多学科协作模式，并推动少见类型甲状腺恶性肿瘤全程规范化诊治体系的构建。未来研究应聚焦于开展更大规模、更高质量的临床研究，以深入阐明其临床特征，探讨靶向药物的应答与耐药机制，推动高选择性激酶抑制剂及免疫治疗药物的研发，从而提升疗效。
Abstract
Rare thyroid malignancies ［including anaplastic thyroid carcinoma (ATC) , medullary thyroid carcinoma (MTC), primary thyroid lymphoma (PTL), primary squamous cell carcinoma of thyroid (PSCCT), poorly differentiated thyroid carcinoma (PDTC) , and primary thyroid sarcoma (PTS)］constitute less than 10% of all thyroid malignancies but account for over 50% of thyroid cancer-related deaths, posing a major clinical challenge. These malignancies are characterized by high aggressiveness, diagnostic difficulty, high rates of metastasis at initial presentation, limited treatment options, and poor prognosis. The key challenges in current management include diagnostic delays, inaccurate staging, and a lack of standardized treatment guidelines. Although the prognosis for many patients has improved in recent years with the advent of targeted drugs and innovations in surgical techniques and concepts, the overall survival rate has not shown significant improvement. To address the diagnosis and treatment of these patients, it is imperative to establish a surgeon-led multidisciplinary team (MDT) approach and a comprehensive standardized management system throughout the entire diagnosis and treatment process for rare thyroid malignancies. Future efforts should focus on conducting larger-scale, higher-quality clinical research to acquire a deeper understanding of the clinical characteristics of these diseases, investigate the response and resistance mechanisms of targeted therapies, and promote the development of highly selective kinase inhibitors and immunotherapeutic agents, thereby improving treatment outcomes for patients with rare thyroid malignancies.
关键词
少见类型甲状腺恶性肿瘤 /
甲状腺未分化癌 /
甲状腺髓样癌 /
规范化诊治 /
多学科协作模式 /
靶向治疗
Key words
rare thyroid malignancies /
anaplastic thyroid carcinoma /
medullary thyroid carcinoma /
standardized diagnosis and treatment /
multidisciplinary team /
targeted therapy
田 文, 刘军言.
重视少见类型甲状腺恶性肿瘤的规范化诊治[J]. 中国实用外科杂志. 2025, 45(09): 979-984 https://doi.org/10.19538/j.cjps.issn1005-2208.2025.09.03
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