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World J Clin Oncol. Dec 24, 2023; 14(12): 570-583
Published online Dec 24, 2023. doi: 10.5306/wjco.v14.i12.570
Update on current diagnosis and management of anaplastic thyroid carcinoma
Efstathios T Pavlidis, Ioannis N Galanis, Theodoros E Pavlidis
Efstathios T Pavlidis, Ioannis N Galanis, Theodoros E Pavlidis, 2nd Propedeutic Department of Surgery, Hippokration General Hospital, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki 54642, Greece
Author contributions: Pavlidis ET, Galanis IN, and Pavlidis TE analyzed data, and reviewed; Pavlidis TE designed research, contributed new analytic tools; Pavlidis ET performed research and wrote the paper.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Theodoros E Pavlidis, Doctor, PhD, Emeritus Professor, Surgeon, 2nd Propedeutic Department of Surgery, Hippokration General Hospital, School of Medicine, Aristotle University of Thessaloniki, Konstantinoupoleos 49, Thessaloniki 54642, Greece. pavlidth@auth.gr
Received: October 5, 2023
Peer-review started: October 5, 2023
First decision: November 9, 2023
Revised: November 9, 2023
Accepted: December 1, 2023
Article in press: December 1, 2023
Published online: December 24, 2023
Processing time: 77 Days and 19.1 Hours
Abstract

Well-differentiated thyroid carcinoma has a favorable prognosis with a 5-year survival rate of over 95%. However, the undifferentiated or anaplastic type accounting for < 0.2%, usually in elderly individuals, exhibits a dismal prognosis with rapid growth and disappointing outcomes. It is the most aggressive form of thyroid carcinoma, with a median survival of 5 mo and poor quality of life (airway obstruction, dysphagia, hoarseness, persistent pain). Early diagnosis and staging are crucial. Diagnostic tools include biopsy (fine needle aspiration, core needle, open surgery), high-resolution ultrasound, computed tomography, magnetic resonance imaging, [(18)F]fluoro-D-glucose positron emission tomo-graphy/computed tomography, liquid biopsy and microRNAs. The BRAF gene (BRAF-V600E and BRAF wild type) is the most often found molecular factor. Others include the genes RET, KRAS, HRAS, and NRAS. Recent management policy is based on surgery, even debulking, chemotherapy (cisplatin or doxorubicin), radiotherapy (adjuvant or definitive), targeted biological agents and immunotherapy. The last two options constitute novel hopeful management modalities improving the overall survival in these otherwise condemned patients. Anti-programmed death-ligand 1 antibody immunotherapy, stem cell targeted therapies, nanotechnology achievements and artificial intelligence imple-mentation provide novel promising alternatives. Genetic mutations determine molecular pathways, thus indicating novel treatment strategies such as anti-BRAF, anti-vascular endothelial growth factor-A, and anti-epidermal growth factor receptor. Treatment with the combination of the BRAF inhibitor dabrafenib and the MEK inhibitor trametinib has been approved by the Food and Drug Administration in cases with BRAF-V600E gene mutations and is currently the standard care. This neoadjuvant treatment followed by surgery ensures a two-year overall survival of 80%. Prognostic factors for improved outcomes have been found to be younger age, earlier tumor stage and radiation therapy. A multidisciplinary approach is necessary, and the therapeutic plan should be individualized based on surveillance and epidemiology end results.

Keywords: Thyroid diseases; Thyroid cancers; Anaplastic carcinoma; Undifferentiated carcinoma; Neck mass; Aggressive malignancies

Core Tip: Anaplastic thyroid carcinoma is uncommon but one of the most lethal neoplasms. The optimal management remains unclear. The addition of novel targeted therapy and immunotherapy to the traditional management of surgery, radiation and chemotherapy has improved the outcomes. Multimodality management and the emerging use of individualized treatment based on novel therapeutic agents offers promising results. However, further research efforts involving the molecular microenvironment and biological drivers should be made.