ESPEYB25 8. Adrenals Important for Clinical Practice (5 abstracts)
8.4. International consensus statement on the diagnosis and management of phaeochromocytoma and paraganglioma in children and adolescents
Casey RT , Hendriks E , Deal C , Waguespack SG , Wiegering V , Redlich A , Akker S , Prasad R , Fassnacht M , Clifton-Bligh R , Amar L , Bornstein S , Canu L , Charmandari E , Chrisoulidou A , Freixes MC , de Krijger R , de Sanctis L , Fojo A , Ghia AJ , Huebner A , Kosmoliaptsis V , Kuhlen M , Raffaelli M , Lussey-Lepoutre C , Marks SD , Nilubol N , Parasiliti-Caprino M , Timmers HHJLM , Zietlow AL , Robledo M , Gimenez-Roqueplo AP , Grossman AB , Taïeb D , Maher ER , Lenders JWM , Eisenhofer G , Jimenez C , Pacak K & Pamporaki C
Nat Rev Endocrinol. 2024; 20(12): 729-748. PMID: 39147856 doi: 10.1038/s41574-024-01024-5. https://pubmed.ncbi.nlm.nih.gov/39147856/
Brief summary: This article presents newinternational guidelines on Pheochromocytomas and Paragangliomas.
Comment: Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors arising from the adrenal medulla or extra-adrenal sympathetic and parasympathetic paraganglia. PPGL are more frequently diagnosed in adult than in pediatric populations; pediatric cases account for only 10–20% of all detected cases of PPGL with an estimated annual incidence of 0.5–2.0 per million children (1). Due to the slow-growing nature of the tumors and usual delays in diagnosis, the true prevalence of PPGL in childhood is likely to be much higher than currently estimated. The median age at presentation is 11–15 years (2, 3) and PPGL are exceedingly rare in children under 5 years of age.
Up to 70–80% of childhood PPGL are hereditary, accounting for a higher incidence of metastatic and/or multifocal PPGL in pediatric patients than in adult patients (4). Key differences in the tumor biology and management, together with rare disease incidence and therapeutic challenges in pediatric compared with adult patients, mandate close expert cross-disciplinary teamwork. Teams should ideally include adult and pediatric endocrinologists, oncologists, cardiologists, surgeons, geneticists, pathologists, radiologists, clinical psychologists and nuclear medicine physicians. This Consensus Statement serves as a catalyst to further promote close working relationships between pediatric and adult specialists managing patients with PPGL, and between specialists and national and international patient support and advocacy groups.
This International Consensus Statement highlights the strong hereditary basis of PPGL and the need for surveillance of asymptomatic genetic carriers from childhood or early adulthood and for lifelong follow-up. In addition, it supports a role for wide-scale adoption of ‘family clinic’ models for families affected by PPGL or families with individuals carrying a PPGL predisposition gene. Finally, this international and collaborative work emphasizes the need for novel treatment options and the need for children and young adults to be included in local, national and international data registries of PPGL and in the design of clinical trials.
References: 1. Bausch B et al. Long-term prognosis of patients with pediatric pheochromocytoma. Endocr. Relat. Cancer 2014: 21: 17–25.2. Barontini M, Levin G, Sanso G. Characteristics of pheochromocytoma in a 4- to 20-year-old population. Ann. N. Y. Acad. Sci. 2006; 1073: 30–37.3. Pamporaki C et al. Characteristics of pediatric vs adult pheochromocytomas and paragangliomas. J. Clin. Endocrinol. Metab. 2017; 102: 1122–1132.4. Neumann HPH. et al. Germ-line mutations in nonsyndromic pheochromocytoma. N. Engl. J. Med. 2002; 346: 1459–1466.
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ESPE Yearbook of Paediatric Endocrinology
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