International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents

IF 3.7 3区 医学 Q2 CHEMISTRY, MEDICINAL
Ruth T. Casey, Emile Hendriks, Cheri Deal, Steven G. Waguespack, Verena Wiegering, Antje Redlich, Scott Akker, Rathi Prasad, Martin Fassnacht, Roderick Clifton-Bligh, Laurence Amar, Stefan Bornstein, Letizia Canu, Evangelia Charmandari, Alexandra Chrisoulidou, Maria Currás Freixes, Ronald de Krijger, Luisa de Sanctis, Antonio Fojo, Amol J. Ghia, Angela Huebner, Vasilis Kosmoliaptsis, Michaela Kuhlen, Marco Raffaelli, Charlotte Lussey-Lepoutre, Stephen D. Marks, Naris Nilubol, Mirko Parasiliti-Caprino, Henri H.J.L.M. Timmers, Anna Lena Zietlow, Mercedes Robledo, Anne-Paule Gimenez-Roqueplo, Ashley B. Grossman, David Taïeb, Eamonn R. Maher, Jacques W. M. Lenders, Graeme Eisenhofer, Camilo Jimenez, Karel Pacak, Christina Pamporaki
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Abstract

Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumours that arise not only in adulthood but also in childhood and adolescence. Up to 70–80% of childhood PPGL are hereditary, accounting for a higher incidence of metastatic and/or multifocal PPGL in paediatric patients than in adult patients. Key differences in the tumour biology and management, together with rare disease incidence and therapeutic challenges in paediatric compared with adult patients, mandate close expert cross-disciplinary teamwork. Teams should ideally include adult and paediatric endocrinologists, oncologists, cardiologists, surgeons, geneticists, pathologists, radiologists, clinical psychologists and nuclear medicine physicians. Provision of an international Consensus Statement should improve care and outcomes for children and adolescents with these tumours. Phaeochromocytomas and paragangliomas (PPGL) are rare endocrine tumours that can affect paediatric patients as well as adults. In this first international Consensus Statement on PPGL in paediatric patients, the authors discuss the diagnosis, management and long-term surveillance of these tumours in children and adolescents.

Abstract Image

Abstract Image

关于儿童和青少年嗜铬细胞瘤和副神经节瘤诊断和管理的国际共识声明
辉铬细胞瘤和副神经节瘤(PPGL)是一种罕见的神经内分泌肿瘤,不仅发生在成年期,也发生在儿童和青少年时期。多达 70-80% 的儿童 PPGL 具有遗传性,因此儿童患者中转移性和/或多灶性 PPGL 的发病率高于成人患者。与成人患者相比,儿科患者在肿瘤生物学和管理方面存在重大差异,加上罕见疾病的发病率和治疗方面的挑战,因此需要跨学科的专家团队密切合作。团队成员最好包括成人和儿童内分泌专家、肿瘤专家、心脏病专家、外科医生、遗传学家、病理学家、放射专家、临床心理学家和核医学医生。提供一份国际共识声明应能改善对患有此类肿瘤的儿童和青少年的护理和治疗效果。
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来源期刊
CiteScore
7.90
自引率
7.30%
发文量
215
审稿时长
3.5 months
期刊介绍: Chemical Research in Toxicology publishes Articles, Rapid Reports, Chemical Profiles, Reviews, Perspectives, Letters to the Editor, and ToxWatch on a wide range of topics in Toxicology that inform a chemical and molecular understanding and capacity to predict biological outcomes on the basis of structures and processes. The overarching goal of activities reported in the Journal are to provide knowledge and innovative approaches needed to promote intelligent solutions for human safety and ecosystem preservation. The journal emphasizes insight concerning mechanisms of toxicity over phenomenological observations. It upholds rigorous chemical, physical and mathematical standards for characterization and application of modern techniques.
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