REVIEW   Open access

Panminerva Medica 2024 Apr 05

DOI: 10.23736/S0031-0808.23.05040-1

Copyright © 2023 THE AUTHORS

This is an open access article distributed under the terms of the CC BY-NC 4.0 license which allows users to distribute, remix, adapt and build upon the manuscript, as long as this is not done for commercial purposes, the user gives appropriate credits to the original author(s) and the source (with a link to the formal publication through the relevant DOI), provides a link to the license and indicates if changes were made.

language: English

Poorly differentiated thyroid carcinoma: molecular, clinico-pathological hallmarks and therapeutic perspectives

Valentina CIRELLO ^{1, 2}, Carla GAMBALE ³, Alyaksandr V. NIKITSKI ⁴, Chie MASAKI ⁵, João ROQUE ⁶, Carla COLOMBO ^{1, 2} ✉

¹ Endocrine Oncology Unit, Department of Endocrine and Metabolic Diseases, IRCCS Istituto Auxologico Italiano, Milan, Italy; ² Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy; ³ Department of Clinical and Experimental Medicine, Endocrine Unit, University Hospital of Pisa, Pisa, Italy; ⁴ Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA; ⁵ Department of Surgery, Ito Hospital, Tokyo, Japan; ⁶ Department of Endocrinology, Diabetes and Metabolism, Centro Hospitalar Universitário Lisboa Norte, Hospital de Santa Maria, Lisbon, Portugal

Poorly differentiated thyroid carcinoma (PDTC) is a rare and extremely aggressive tumor, accounting for about 2-15% of all thyroid cancer. PDTC has a distinct biological behavior compared to well-differentiated and anaplastic thyroid carcinoma and, in last years, it has been classified as a separate entity from both anatomopathological and clinical points of view. Nevertheless, there is still a lack of consensus among clinicians regarding inclusion criteria and definition of PDTC that affects its diagnosis and clinical management. Due to its rarity and difficulty in classification compared to other tumors, very few studies are available to date and series often include different histotypes in addition to PDTC. This review focuses on main studies concerning PDTC summarizing the evolution in the definition of its diagnosis criteria, clinicopathological features, management, and outcome. The data available confirm that the pathological evaluation and classification of PDTC are crucial and should therefore be standardized. Since the clinical presentation and prognosis of PDTC may vary widely depending on the different stage of the disease at diagnosis, the patient’s management may differ in treatment and should be tailored to each patient. Finally, this review discusses advances in molecular insights of PDTC that, together with the implementation of both in vitro and in vivo models, will provide valuable insights into biological mechanisms of progression, metastasis, and invasion of this aggressive thyroid carcinoma. Further studies on larger, carefully selected series are needed to better assess the peculiar features of PDTC and to better define its management by focusing on the best diagnostic and therapeutic approaches.

KEY WORDS: Thyroid neoplasms; Tyrosine kinase inhibitors; Prognosis