Pheochromocytoma and Paraganglioma in Children and Adolescents: Experience of the French Society of Pediatric Oncology (SFCE) - Aix-Marseille Université Access content directly
Journal Articles Journal of the Endocrine Society Year : 2020

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

1 IGR - Institut Gustave Roussy
2 Département de cancérologie de l'enfant et de l'adolescent [Gustave Roussy]
3 IHOPe - Institut d'hématologie et d'oncologie pédiatrique [CHU - HCL]
4 CHUGA - Centre Hospitalier Universitaire [CHU Grenoble]
5 Université Paris-Saclay
6 MMG - Marseille medical genetics - Centre de génétique médicale de Marseille
7 Laboratoire de Biochimie et de Biologie Moléculaire [Hôpital de la Conception - APHM]
8 Unité d'hémato-immuno-oncologie pédiatrique [CHU Caen]
9 UNICANCER/Lille - Centre Régional de Lutte contre le Cancer Oscar Lambret [Lille]
10 Service d'Hématologie et Oncologie pédiatriques [CHU Strasbourg]
11 CHU de Bordeaux Pellegrin [Bordeaux]
12 Hôpital Necker - Enfants Malades [AP-HP]
13 Hôpital Sud [CHU Rennes]
14 EPICEA [CRESS - U1153 / UMR_A 1125] - Epidemiology of childhood and adolescent cancer | Epidémiologie des cancers de l'enfant et de l'adolescent
15 CHRU Nancy - Centre Hospitalier Régional Universitaire de Nancy
16 Centre de recherche de l'Institut Curie [Paris]
17 SFCE - Société française du cancer de l'enfant [Rennes]
18 Hôpital universitaire Robert Debré [Reims]
19 Département d'hématologie et oncologie pédiatrique [CHU La Réunion]
20 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
21 CHU ST-E - Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne]
22 Département de pédiatrie [CHU Nantes]
23 PARCC (UMR_S 970/ U970) - Paris-Centre de Recherche Cardiovasculaire
24 HEGP - Hôpital Européen Georges Pompidou [APHP]
Virginie Gandemer
Sabine Sarnacki


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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hal-03222589 , version 1 (04-07-2023)


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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, 2020, 4 (5), ⟨10.1210/jendso/bvaa039⟩. ⟨hal-03222589⟩
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