Background: Adrenal (formerly termed pheochromocytomas) and extra-adrenal paragangliomas (PGLs) in children and adolescents are rare neuroendocrine tumours characterized by unique biological behaviour, a strong hereditary component, and significant risk of recurrence and metastatic progression. Their management requires specialized, multidisciplinary care. Objective: This guidance provides harmonized, evidence-graded recommendations for the diagnosis, treatment, and follow-up of paediatric PGL, developed by the European Cooperative Study Group for Paediatric Rare Tumours (EXPeRT) as part of the European Standard Clinical Practice (ESCP) initiative. Methods: Recommendations were formulated through structured consensus by a multidisciplinary panel of experts in paediatric oncology, endocrinology, surgery, nuclear medicine, genetics, and pathology, based on literature review, existing international guidelines, and integration of external adviser feedback. Levels of evidence and grades of recommendation follow a simplified GRADE (Grading of Recommendations, Assessment, Development and Evaluation) system. Recommendations: Diagnosis should be based on plasma-free metanephrine and normetanephrine as first-line test, complemented by appropriate anatomical and functional imaging guided by biochemical phenotype and genotype. Germline genetic testing is recommended. Tumour resection remains the mainstay of curative treatment, with cortical-sparing adrenalectomy advised in bilateral hereditary cases (except SDHB). Management of metastatic disease should be individualized, incorporating radionuclide therapy, systemic treatments, focal therapies, and palliative care as appropriate. Lifelong surveillance is essential, tailored to genotype and disease characteristics. Conclusion: This European clinical guidance offers practical recommendations to support multidisciplinary management of paediatric PGL within European healthcare systems, complementing existing international consensus statements and supporting harmonization of care.
European clinical guidance for the management of adrenal and extra-adrenal paraganglioma in children and adolescents: a consensus by the EXPeRT group
Virgone, Calogero;Bisogno, Gianni;
2025
Abstract
Background: Adrenal (formerly termed pheochromocytomas) and extra-adrenal paragangliomas (PGLs) in children and adolescents are rare neuroendocrine tumours characterized by unique biological behaviour, a strong hereditary component, and significant risk of recurrence and metastatic progression. Their management requires specialized, multidisciplinary care. Objective: This guidance provides harmonized, evidence-graded recommendations for the diagnosis, treatment, and follow-up of paediatric PGL, developed by the European Cooperative Study Group for Paediatric Rare Tumours (EXPeRT) as part of the European Standard Clinical Practice (ESCP) initiative. Methods: Recommendations were formulated through structured consensus by a multidisciplinary panel of experts in paediatric oncology, endocrinology, surgery, nuclear medicine, genetics, and pathology, based on literature review, existing international guidelines, and integration of external adviser feedback. Levels of evidence and grades of recommendation follow a simplified GRADE (Grading of Recommendations, Assessment, Development and Evaluation) system. Recommendations: Diagnosis should be based on plasma-free metanephrine and normetanephrine as first-line test, complemented by appropriate anatomical and functional imaging guided by biochemical phenotype and genotype. Germline genetic testing is recommended. Tumour resection remains the mainstay of curative treatment, with cortical-sparing adrenalectomy advised in bilateral hereditary cases (except SDHB). Management of metastatic disease should be individualized, incorporating radionuclide therapy, systemic treatments, focal therapies, and palliative care as appropriate. Lifelong surveillance is essential, tailored to genotype and disease characteristics. Conclusion: This European clinical guidance offers practical recommendations to support multidisciplinary management of paediatric PGL within European healthcare systems, complementing existing international consensus statements and supporting harmonization of care.
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