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      Medullary Thyroid Carcinoma: An Update on Imaging

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          Abstract

          Medullary thyroid carcinoma (MTC), arising from the parafollicular C cells of the thyroid, accounts for 1–2% of thyroid cancers. MTC is frequently aggressive and metastasizes to cervical and mediastinal lymph nodes, lungs, liver, and bones. Although a number of new imaging modalities for directing the management of oncologic patients evolved over the last two decades, the clinical application of these novel techniques is limited in MTC. In this article, we review the biology and molecular aspects of MTC as an important background for the use of current imaging modalities and approaches for this tumor. We discuss the modern and currently available imaging techniques—advanced magnetic resonance imaging (MRI)-based techniques such as whole-body MRI, dynamic contrast-enhanced (DCE) technique, diffusion-weighted imaging (DWI), positron emission tomography/computed tomography (PET/CT) with 18F-FDOPA and 18F-FDG, and integrated positron emission tomography/magnetic resonance (PET/MR) hybrid imaging—for primary as well as metastatic MTC tumor, including its metastatic spread to lymph nodes and the most common sites of distant metastases: lungs, liver, and bones.

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          Most cited references86

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          Vandetanib in patients with locally advanced or metastatic medullary thyroid cancer: a randomized, double-blind phase III trial.

          There is no effective therapy for patients with advanced medullary thyroid carcinoma (MTC). Vandetanib, a once-daily oral inhibitor of RET kinase, vascular endothelial growth factor receptor, and epidermal growth factor receptor signaling, has previously shown antitumor activity in a phase II study of patients with advanced hereditary MTC. Patients with advanced MTC were randomly assigned in a 2:1 ratio to receive vandetanib 300 mg/d or placebo. On objective disease progression, patients could elect to receive open-label vandetanib. The primary end point was progression-free survival (PFS), determined by independent central Response Evaluation Criteria in Solid Tumors (RECIST) assessments. Between December 2006 and November 2007, 331 patients (mean age, 52 years; 90% sporadic; 95% metastatic) were randomly assigned to receive vandetanib (231) or placebo (100). At data cutoff (July 2009; median follow-up, 24 months), 37% of patients had progressed and 15% had died. The study met its primary objective of PFS prolongation with vandetanib versus placebo (hazard ratio [HR], 0.46; 95% CI, 0.31 to 0.69; P < .001). Statistically significant advantages for vandetanib were also seen for objective response rate (P < .001), disease control rate (P = .001), and biochemical response (P < .001). Overall survival data were immature at data cutoff (HR, 0.89; 95% CI, 0.48 to 1.65). A final survival analysis will take place when 50% of the patients have died. Common adverse events (any grade) occurred more frequently with vandetanib compared with placebo, including diarrhea (56% v 26%), rash (45% v 11%), nausea (33% v 16%), hypertension (32% v 5%), and headache (26% v 9%). Vandetanib demonstrated therapeutic efficacy in a phase III trial of patients with advanced MTC (ClinicalTrials.gov NCT00410761).
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            Thyroid Ultrasound Reporting Lexicon: White Paper of the ACR Thyroid Imaging, Reporting and Data System (TIRADS) Committee.

            Ultrasound is the most commonly used imaging technique for the evaluation of thyroid nodules. Sonographic findings are often not specific, and definitive diagnosis is usually made through fine-needle aspiration biopsy or even surgery. In reviewing the literature, terms used to describe nodules are often poorly defined and inconsistently applied. Several authors have recently described a standardized risk stratification system called the Thyroid Imaging, Reporting and Data System (TIRADS), modeled on the BI-RADS system for breast imaging. However, most of these TIRADS classifications have come from individual institutions, and none has been widely adopted in the United States. Under the auspices of the ACR, a committee was organized to develop TIRADS. The eventual goal is to provide practitioners with evidence-based recommendations for the management of thyroid nodules on the basis of a set of well-defined sonographic features or terms that can be applied to every lesion. Terms were chosen on the basis of demonstration of consistency with regard to performance in the diagnosis of thyroid cancer or, conversely, classifying a nodule as benign and avoiding follow-up. The initial portion of this project was aimed at standardizing the diagnostic approach to thyroid nodules with regard to terminology through the development of a lexicon. This white paper describes the consensus process and the resultant lexicon.
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              A Phase II Trial of the Multitargeted Tyrosine Kinase Inhibitor Lenvatinib (E7080) in Advanced Medullary Thyroid Cancer.

              Positive results of phase I studies evaluating lenvatinib in solid tumors, including thyroid cancer, prompted a phase II trial in advanced medullary thyroid carcinoma (MTC).
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                Author and article information

                Contributors
                Journal
                J Thyroid Res
                J Thyroid Res
                JTR
                Journal of Thyroid Research
                Hindawi
                2090-8067
                2042-0072
                2019
                7 July 2019
                : 2019
                : 1893047
                Affiliations
                1Moffitt Cancer Center, Department of Radiology, 12902 USF Magnolia Drive, Tampa, FL 33612, USA
                2Johns Hopkins Hospital, Department of Radiology, 601 N Caroline St, Baltimore, MD 21287, USA
                3Diabetes, Endocrinology, and Obesity Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda 10, Center Dr, Bethesda, MD 20892, USA
                4Department of Medicine, Division of Endocrinology, Diabetes and Metabolism, University of South Carolina School of Medicine, 6311 Garners Ferry Rd, Columbia, SC, USA
                5Department of Radiology, Mercy Catholic Medical Center, 1500 Lansdowne Ave, Darby, PA 19023, USA
                6Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD/NIH), 10 Center Dr, Bethesda, MD 20892, USA
                Author notes

                Academic Editor: Noriyuki Koibuchi

                Author information
                https://orcid.org/0000-0002-1373-5255
                https://orcid.org/0000-0001-8878-1283
                Article
                10.1155/2019/1893047
                6642782
                31360432
                6e6fc713-0e42-4b6a-b191-f422defbd7e5
                Copyright © 2019 Sergiy V. Kushchayev et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 7 April 2019
                : 8 May 2019
                Funding
                Funded by: National Institutes of Health
                Award ID: Z1AHD008735
                Categories
                Review Article

                Endocrinology & Diabetes
                Endocrinology & Diabetes

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