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      Development of a pediatric differentiated thyroid carcinoma registry within the EuRRECa project: rationale and protocol


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          Although differentiated thyroid carcinoma (DTC) is the most frequent endocrine pediatric cancer, it is rare in childhood and adolescence. While tumor persistence and recurrence are not uncommon, mortality remains extremely low. Complications of treatment are however reported in up to 48% of the survivors. Due to the rarity of the disease, current treatment guidelines are predominantly based on the results of small observational retrospective studies and extrapolations from results in adult patients. In order to develop more personalized treatment and follow-up strategies (aiming to reduce complication rates), there is an unmet need for uniform international prospective data collection and clinical trials.

          Methods and analysis

          The European pediatric thyroid carcinoma registry aims to collect clinical data for all patients ≤18 years of age with a confirmed diagnosis of DTC who have been diagnosed, assessed, or treated at a participating site. This registry will be a component of the wider European Registries for Rare Endocrine Conditions project which has close links to Endo-ERN, the European Reference Network for Rare Endocrine Conditions. A multidisciplinary expert working group was formed to develop a minimal dataset comprising information regarding demographic data, diagnosis, treatment, and outcome. We constructed an umbrella-type registry, with a detailed basic dataset. In the future, this may provide the opportunity for research teams to integrate clinical research questions.

          Ethics and dissemination

          Written informed consent will be obtained from all participants and/or their parents/guardians. Summaries and descriptive analyses of the registry will be disseminated via conference presentations and peer-reviewed publications.

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          Most cited references22

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          The changing incidence of thyroid cancer.

          During the past few decades, the incidence of thyroid cancer has increased substantially in many countries, including the USA. The rise in incidence seems to be attributable both to the growing use of diagnostic imaging and fine-needle aspiration biopsy, which has led to enhanced detection and diagnosis of subclinical thyroid cancers, and environmental factors. The latest American Thyroid Association (ATA) practice guidelines for the management of adult patients with thyroid nodules and differentiated thyroid cancer differ substantially from the previous ATA guidelines published in 2009. Specifically, the problems of overdiagnosis and overtreatment of a disease that is typically indolent, where treatment-related morbidity might not be justified by a survival benefit, now seem to be acknowledged. As few modifiable risk factors for thyroid cancer have been established, the specific environmental factors that have contributed to the rising incidence of thyroid cancer remain speculative. However, the findings of several large, well-designed epidemiological studies have provided new information about exposures (such as obesity) that might influence the development of thyroid cancer. In this Review, we describe the changing incidence of thyroid cancer, suggest potential explanations for these trends, emphasize the implications for patients and highlight ongoing and potential strategies to combat this growing clinical and public health issue.
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            Management Guidelines for Children with Thyroid Nodules and Differentiated Thyroid Cancer.

            Previous guidelines for the management of thyroid nodules and cancers were geared toward adults. Compared with thyroid neoplasms in adults, however, those in the pediatric population exhibit differences in pathophysiology, clinical presentation, and long-term outcomes. Furthermore, therapy that may be recommended for an adult may not be appropriate for a child who is at low risk for death but at higher risk for long-term harm from overly aggressive treatment. For these reasons, unique guidelines for children and adolescents with thyroid tumors are needed.
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              Is Open Access

              Thyroid Cancer in the Pediatric Population

              Thyroid cancer is rare in the pediatric population, but thyroid carcinomas occurring in children carry a unique set of clinical, pathologic, and molecular characteristics. In comparison to adults, children more often present with aggressive, advanced stage disease. This is at least in part due to the underlying biologic and molecular differences between pediatric and adult thyroid cancer. Specifically, papillary thyroid carcinoma (which accounts for approximately 90% of pediatric thyroid cancer) has a high rate of gene fusions which influence the histologic subtypes encountered in pediatric thyroid tumors, are associated with more extensive extrathyroidal disease, and offer unique options for targeted medical therapies. Differences are also seen in pediatric follicular thyroid cancer, although there are few studies of non-papillary pediatric thyroid tumors published in the literature due to their rarity, and in medullary carcinoma, which is most frequently diagnosed in the pediatric population in the setting of prophylactic thyroidectomies for known multiple endocrine neoplasia syndromes. The overall shift in the spectrum of histotypes and underlying molecular alterations common in pediatric thyroid cancer is important to recognize as it may directly influence diagnostic test selection and therapeutic recommendations.

                Author and article information

                Endocr Connect
                Endocr Connect
                Endocrine Connections
                Bioscientifica Ltd (Bristol )
                10 January 2023
                01 March 2023
                : 12
                : 3
                : e220306
                [1 ]Department of Pediatrics , Emma Children’s Hospital, Amsterdam University Medical Center, University of Amsterdam, Amsterdam, The Netherlands
                [2 ]Department of Pediatric Endocrinology , Wilhelmina Children’s Hospital/ University Medical Center Utrecht, Utrecht, The Netherlands
                [3 ]Academic Center For Thyroid Disease , Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands
                [4 ]Princess Máxima Center for Pediatric Oncology , Utrecht, The Netherlands
                [5 ]Department of Nuclear Medicine , University of Brescia and Spedali Civili of Brescia, Brescia, Italy
                [6 ]Department of Pediatrics , Radboud University Medical Center, Amalia Children's Hospital, Nijmegen, The Netherlands
                [7 ]The Oncologic and Reconstructive Surgery Clinic , M. Sklodowska-Curie National Research Institute of Oncology Gliwice Branch, Gliwice, Poland
                [8 ]Department of Endocrinology and Diabetes , Birmingham Children’s Hospital, Birmingham Women’s, and Children’s NHS Foundation Trust, Birmingham, UK
                [9 ]Institute of Metabolism and Systems Research , College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
                [10 ]Department of Pediatric Endocrinology , Children's Clinical University Hospital, Riga, Latvia
                [11 ]Endocrine Unit , Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
                [12 ]Nuclear Medicine Department , Vall d'Hebron University Hospital, Barcelona, Spain
                [13 ]Department of Clinical Genetics , Guy's and St Thomas’ NHS Foundation Trust, London, UK
                [14 ]Division of Endocrinology , Diabetes, and Metabolism, First Department of Pediatrics National and Kapodistrian University of Athens Medical School, Aghia Sophia Children's Hospital, Athens, Greece
                [15 ]Institute of Experimental Pediatric Endocrinology , Charité - Universitätsmedizin, Berlin, Germany
                [16 ]Department of Endocrine Oncology , Gustave Roussy, Villejuif, France
                [17 ]Department of Visceral , Vascular and Endocrine Surgery, Martin Luther University Halle-Wittenberg, Halle (Saale), Germany
                [18 ]Department of Nuclear Medicine , University Hospital Marburg, Marburg, Germany
                [19 ]Department of Endocrinology , Lithuanian University of Health Sciences, Kaunas, Lithuania
                [20 ]Nuclear Medicine Service - Institut de diagnòstic per la Imatge , Hospital Universitari de Girona Dr. Josep Trueta, Girona, Spain
                [21 ]Thyroid Therapy Unit , The Royal Marsden NHS Foundation Trust Hospital, London, UK
                [22 ]Endocrinology , Endocrinology Division, Garibaldi-Nesima Medical Center, Catania, Italy
                [23 ]Department of Nuclear Medicine , EO Ospedali Galliera, Genoa, Italy
                [24 ]Department of Medicine , Division of Endocrinology, Leiden, University medical Center, Leiden, The Netherlands
                [25 ]Pediatric Oncology Department , Otto von Guericke University Children's Hospital, Magdeburg, Germany
                [26 ]Regina Margherita Children Hospital - Department of Public Health and Pediatric Sciences , University of Torino, Torino, Italy
                [27 ]University Hospital of São João , Medical Faculty and Institute of Molecular Pathology and Immunology, University of Porto, Porto, Portugal
                [28 ]Department of Pediatric Endocrinology , Emma Children’s Hospital, Amsterdam University Medical Center, University of Amsterdam, Amsterdam, The Netherlands
                [29 ]Department of Radiology & Nuclear Medicine , Erasmus MC Rotterdam, Rotterdam, The Netherlands
                [30 ]Department of Endocrine Surgery , University Medical Center Utrecht, Utrecht, The Netherlands
                [31 ]Department of Endocrinology , University Medical Center Groningen, Groningen, The Netherlands
                [32 ]Developmental Endocrinology Research Group , Royal Hospital for Children, University of Glasgow, Glasgow, UK
                [33 ]Office for Rare Conditions , University of Glasgow, Glasgow, UK
                Author notes
                Correspondence should be addressed to H M van Santen: h.m.vansanten@ 123456umcutrecht.nl
                Author information
                © The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                : 30 December 2022
                : 10 January 2023

                registry,dtc,thyroid carcinoma,childhood
                registry, dtc, thyroid carcinoma, childhood


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