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

Marie de Tersant 1, 2 Lucile Généré 3 Claire Freyçon 4 Sophie Villebasse 1, 5 Rachid Abbas 1, 2 Anne Barlier 6, 7 Damien Bodet 8 Nadège Corradini 3 Anne-Sophie Defachelles 9 Natacha Entz-Werle 10 Cyrielle Fouquet 11 Louise Galmiche 12 Virginie Gandemer 13 Brigitte Lacour 14 Ludovic Mansuy 15 Daniel Orbach 16, 17 Claire Pluchart 18 Yves Réguerre 19 Charlotte Rigaud 1, 2 Sabine Sarnacki 12 Nicolas Sirvent 20 Jean-Louis Stephan 21 Estelle Thebaud 22 Anne-Paule Gimenez-Roqueplo 23, 24 Laurence Brugières 1, 2
14 CRESS - U1153 - Equipe 7 : EPICEA - Epidémiologie des cancers de l'enfant et de l'adolescent
CRESS (U1153 / UMR_A_1125 / UMR_S_1153) - Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité
Abstract : 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. 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. 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). Conclusions Most pediatric PPGLs are associated with germline mutations and require specific follow-up because of the high risk of tumor recurrence.
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Submitted on : Monday, May 10, 2021 - 12:53:33 PM
Last modification on : Monday, May 2, 2022 - 3:42:54 PM

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Marie de Tersant, Lucile Généré, Claire Freyçon, Sophie Villebasse, Rachid Abbas, et al.. Pheochromocytoma and Paraganglioma in Children and Adolescents: Experience of the French Society of Pediatric Oncology (SFCE). Journal of the Endocrine Society, Oxford University Press, 2020, 4 (5), ⟨10.1210/jendso/bvaa039⟩. ⟨hal-03222589⟩

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