甲状腺未分化癌（ATC）是一种罕见且高度侵袭性的恶性肿瘤，其特点是预后极差，大多数患者表现为广泛的局部浸润和/或远处转移。 20-30% 的 ATC 带有 BRAF-V600E 突变。新辅助 BRAF 靶向治疗可能有潜力使患有 BRAF 突变的局部晚期且不可切除的原发性肿瘤患者降期并促进手术切除，并可能为患有转移性疾病的患者带来生存优势。越来越多的证据支持使用其他靶向药物，包括多激酶抑制剂，以及将免疫疗法纳入治疗方案。快速的分子和病​​理诊断以及专业治疗中心的专家多学科讨论对于加快这种复杂且快速进展的疾病的调查和启动治疗至关重要。© 2024 作者。约翰·威利 (John Wiley) 出版的《亚太临床肿瘤学杂志》

Anaplastic thyroid cancer (ATC), a rare and highly aggressive malignancy, is characterized by an exceptionally poor prognosis, where the majority of patients present with extensive local invasion and/or distant metastases. 20-30% of ATCs harbor the BRAF-V600E mutation. Neoadjuvant BRAF-targeted therapy may have the potential to downstage and facilitate surgical resection for patients with locally advanced and unresectable primary tumors with BRAF mutation and may convey a survival advantage in those with metastatic disease. There is emerging evidence to support the use of other targeted agents, including multikinase inhibitors, as well as the incorporation of immunotherapy into the treatment regimen. Rapid molecular and pathological diagnosis and expert multidisciplinary discussion at specialized treatment centers are critical to expedite investigations and initiate treatment for this complex and rapidly progressive disease.© 2024 The Author(s). Asia‐Pacific Journal of Clinical Oncology published by John Wiley & Sons Australia, Ltd.