Pattern of loco‐regional relapses and treatment in pediatric esthesioneuroblastoma: The French very rare tumors group ( Fracture ) contribution
2020
Dumont, Benoît | Fresneau, Brice | Claude, Line | Defachelles, Anne‐sophie | Couloigner, Vincent | Puget, Stéphanie | Brisse, Hervé | Fréneaux, Paul | Lacour, Brigitte | Orbach, Daniel | Institut Curie [Paris] | Centre de recherche en épidémiologie et santé des populations (CESP) ; Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay | Institut Gustave Roussy (IGR) | Centre Léon Bérard [Lyon] | Centre Oscar Lambert | Hôpital Necker - Enfants Malades [AP-HP] ; Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP) | Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163) ; Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité) | Département de Biologie des Tumeurs ; Institut Curie [Paris] | Epidemiology of childhood and adolescent cancer | Epidémiologie des cancers de l'enfant et de l'adolescent (EPICEA [CRESS - U1153 / UMR_A 1125]) ; Centre for Research in Epidemiology and Statistics | Centre de Recherche Épidémiologie et Statistiques (CRESS (U1153 / UMR_A 1125)) ; Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE) | Registre National des Tumeurs Solides de l'Enfant (RNTSE) ; Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy) | Registre National des Cancers de l'Enfant - National Registry of Childhood Cancers [Villejuif & CHU Nancy] (RNCE) ; Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)
International audience
Show more [+] Less [-]English. Background: Esthesioneuroblastoma (ENB) is a rare neuroectodermal tumor that seldom occurs during childhood. Multimodal treatments are currently proposed, but the place of each therapy is still in debate. Our objective is to describe clinical evolution, especially the pattern of relapses and determine contributors to tumor progression.Procedure: Medical charts of all children (≤18 years) affected by ENB treated in France from January 1990 to December 2015 were retrospectively analyzed.Results: Eighteen patients were selected (10 males). Median age at diagnosis was 12.2 years (0.9-18). Tumor extension was Kadish stage A (n = 1), B (n = 3), C (n = 10), and D (n = 4). Hyams histological grades were I (n = 1), II (n = 3), III (n = 6), and IV (n = 6) (in two cases not defined). Initial cervical nodal spread was assessed by magnetic resonance imaging (n = 15), computed tomography scan (n = 16), fluorodeoxyglucose-positron emission tomography-computed tomography (n = 7), and cytological/histological analysis (n = 2). N1 stage was confirmed by imaging in two of 18 cases and one of two cases had cervical node dissection with neck irradiation (58 Gy). After a median follow-up of survivors of 7.6 years (3.8-17.9), 10 patients developed neuromeningeal progression, whereas no cervical nodal relapse occurred and only eight survived. Both 5-year overall and event-free survival rates were 44.4% (±11.7%).Conclusions: The poor prognosis is mainly related to neuromeningeal dissemination that should be considered during treatment strategy. However, cervical lymph node relapse is rare.
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