Latest Developments in Anaplastic Thyroid Carcinoma Management in 2024

Anaplastic thyroid carcinomas (ATCs) represent a rare and undifferentiated form of thyroid cancer with a poor prognosis, typically marked by a median overall survival of four to ten months. However, recent advances have shown improvements due to the more systematic application of molecular testing, targeted therapies, and immunotherapy, alongside the establishment of rapid specialized care protocols in expert centers. Clinically, ATCs often present as a rapidly enlarging cervical mass originating from the thyroid, causing neck, pain and tenderness, dyspnea and dysphagia, and associated lymphadenopathy, typically in elderly patients. Diagnostic confirmation requires an urgent biopsy, reviewed by a pathologist within the TUTHYREF-path network, with a mandatory search for BRAF(V600E) molecular alterations. Following diagnosis, care coordination is expedited within an expert center of the ENDOCAN-TUTHYREF network whenever feasible. Initial surgery is rarely an option due to frequent loco-regional cervical invasion. A multimodal treatment approach is essential: BRAF/MEK inhibitors (dabrafenib/trametinib) in cases of BRAF(V600E) mutation, or chemotherapy in the absence of a molecular target, combined with radiotherapy and, potentially, surgery if the disease becomes resectable following induction therapy. PD-1/PD-L1 targeted immunotherapy, either alone or in combination with targeted therapies, has shown potential to extend survival in some patients; however, predictive biomarkers and the optimal sequencing of immunotherapy (whether as induction and/or maintenance) require further clarification and may vary depending on the clinical context.