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

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

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Attribution - NonCommercial - NoDerivatives

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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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