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.
Key words
rare thyroid malignancies /
anaplastic thyroid carcinoma /
medullary thyroid carcinoma /
standardized diagnosis and treatment /
multidisciplinary team /
targeted therapy
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