Back to Search
Start Over
Pheochromocytoma and Paraganglioma in Children and Adolescents: Experience of the French Society of Pediatric Oncology (SFCE).
- Source :
-
Journal of the Endocrine Society [J Endocr Soc] 2020 Apr 03; Vol. 4 (5), pp. bvaa039. Date of Electronic Publication: 2020 Apr 03 (Print Publication: 2020). - Publication Year :
- 2020
-
Abstract
- Purpose: 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.<br />Methods: 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.<br />Results: 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 ( SDHB [n = 25], VHL [n = 21], RET [n = 2], HIF2A [n = 2], SDHC [n = 1], SDHD [n = 1], NF1 [n = 1]). Incomplete resection and synchronous metastases were associated with higher risk of events ( P = .011, P = .004), but presence of a germline mutation was not ( P = .11).<br />Conclusions: Most pediatric PPGLs are associated with germline mutations and require specific follow-up because of the high risk of tumor recurrence.<br /> (© Endocrine Society 2020.)
Details
- Language :
- English
- ISSN :
- 2472-1972
- Volume :
- 4
- Issue :
- 5
- Database :
- MEDLINE
- Journal :
- Journal of the Endocrine Society
- Publication Type :
- Academic Journal
- Accession number :
- 32432211
- Full Text :
- https://doi.org/10.1210/jendso/bvaa039