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Pheochromocytoma and Paraganglioma in Children and Adolescents: Experience of the French Society of Pediatric Oncology (SFCE).

Authors :
de Tersant M
Généré L
Freyçon C
Villebasse S
Abbas R
Barlier A
Bodet D
Corradini N
Defachelles AS
Entz-Werle N
Fouquet C
Galmiche L
Gandemer V
Lacour B
Mansuy L
Orbach D
Pluchart C
Réguerre Y
Rigaud C
Sarnacki S
Sirvent N
Stephan JL
Thebaud E
Gimenez-Roqueplo AP
Brugières L
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