Pheochromocytoma and Paraganglioma in Children and Adolescents: Experience of the French Society of Pediatric Oncology (SFCE).
SDHB
genetic
paraganglioma
pediatric
pheochromocytoma
Journal
Journal of the Endocrine Society
ISSN: 2472-1972
Titre abrégé: J Endocr Soc
Pays: United States
ID NLM: 101697997
Informations de publication
Date de publication:
01 May 2020
01 May 2020
Historique:
received:
25
08
2019
accepted:
01
04
2020
entrez:
21
5
2020
pubmed:
21
5
2020
medline:
21
5
2020
Statut:
epublish
Résumé
The purpose of this work is to assess the clinical outcome of pediatric patients diagnosed with pheochromocytoma and paraganglioma (PPGL) detected in France since 2000. A retrospective multicenter study was conducted that included all patients younger than 18 years with PPGL diagnosed in France between 2000 and 2016. Patients were identified from 4 different sources: the National Registry of Childhood Solid Tumors, the French Pediatric Rare Tumors Database, the French registry of succinate dehydrogenase (SDH)-related hereditary paraganglioma, and the nationwide TenGen network. Among 113 eligible patients, 81 children with available data were enrolled (41 with adrenal and 40 with extra-adrenal PPGL). At diagnosis, 11 had synchronous metastases. After a median follow-up of 53 months, 27 patients experienced a new event (n = 7 second PPGL, n = 1 second paraganglioma [PGL], n = 8 local recurrences, n = 10 metastatic relapses, n = 1 new tumor) and 2 patients died of their disease. The 3- and 10-year event-free survival rates were 80% (71%-90%) and 39% (20%-57%),respectively, whereas the overall survival rate was 97% (93%-100%)at 3 and 10 years. A germline mutation in one PPGL-susceptibility gene was identified in 53 of the 68 (77%) patients who underwent genetic testing ( Most pediatric PPGLs are associated with germline mutations and require specific follow-up because of the high risk of tumor recurrence.
Identifiants
pubmed: 32432211
doi: 10.1210/jendso/bvaa039
pii: bvaa039
pmc: PMC7217277
doi:
Types de publication
Journal Article
Langues
eng
Pagination
bvaa039Informations de copyright
© Endocrine Society 2020.
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