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International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents
Nature Reviews Endocrinology ( IF 31.0 ) Pub Date : 2024-08-15 , DOI: 10.1038/s41574-024-01024-5
Ruth T Casey 1, 2 , Emile Hendriks 3 , Cheri Deal 4 , Steven G Waguespack 5 , Verena Wiegering 6 , Antje Redlich 7 , Scott Akker 8 , Rathi Prasad 9 , Martin Fassnacht 10 , Roderick Clifton-Bligh 11 , Laurence Amar 12, 13 , Stefan Bornstein 14 , Letizia Canu 15, 16 , Evangelia Charmandari 17 , Alexandra Chrisoulidou 18 , Maria Currás Freixes 19 , Ronald de Krijger 20, 21 , Luisa de Sanctis 22 , Antonio Fojo 23 , Amol J Ghia 24 , Angela Huebner 25 , Vasilis Kosmoliaptsis 26, 27 , Michaela Kuhlen 28 , Marco Raffaelli 29, 30 , Charlotte Lussey-Lepoutre 31 , Stephen D Marks 32 , Naris Nilubol 33 , Mirko Parasiliti-Caprino 34 , Henri H J L M Timmers 35 , Anna Lena Zietlow 36 , Mercedes Robledo 19 , Anne-Paule Gimenez-Roqueplo 37, 38 , Ashley B Grossman 39, 40, 41 , David Taïeb 42 , Eamonn R Maher 1 , Jacques W M Lenders 35 , Graeme Eisenhofer 14 , Camilo Jimenez 5 , Karel Pacak 43 , Christina Pamporaki 14
Affiliation  

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.



中文翻译:


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



嗜铬细胞瘤和副神经节瘤 (PPGL) 是罕见的神经内分泌肿瘤,不仅出现在成年期,也出现在儿童期和青少年期。高达 70-80% 的儿童 PPGL 是遗传性的,导致儿科患者转移性和/或多灶性 PPGL 的发生率高于成人患者。与成人患者相比,儿科在肿瘤生物学和管理方面的主要差异,以及罕见病的发病率和治疗挑战,需要密切的专家跨学科团队合作。理想情况下,团队应包括成人和儿童内分泌学家、肿瘤学家、心脏病专家、外科医生、遗传学家、病理学家、放射科医生、临床心理学家和核医学医师。提供国际共识声明应改善患有这些肿瘤的儿童和青少年的护理和结局。

更新日期:2024-08-15
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