Benoît Dumont1, Brice Fresneau2, Line Claude3, Anne-Sophie Defachelles4, Vincent Couloigner5, Stéphanie Puget6, Hervé J Brisse7, Paul Fréneaux8, Brigitte Lacour9,10, Daniel Orbach1. 1. SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), Institut Curie, PSL University, Paris, France. 2. Department of Pediatric and Adolescent Oncology, Gustave Roussy, Université Paris-Saclay, Villejuif, France. 3. Department of Radiation Oncology, Léon Bérard Center, Lyon, France. 4. Department of Pediatric Oncology, Oscar Lambret Center, Lille, France. 5. Pediatric Head and Neck Surgery and Otorhinolaryngology Department, Necker Enfants-Malades Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. 6. Pediatric Neurosurgery Department, Necker Enfants-Malades Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. 7. Imaging Department, Institut Curie, Paris, France. 8. Department of Biopathology, Institut Curie, Paris, France. 9. National Registry of Childhood Solid Tumors, CHU de Nancy, Vandœuvre-lès-Nancy, France. 10. Inserm U1153, Center of Research in Epidemiology and Statistics (CRESS), Paris University, Epidemiology of Childhood and Adolescent Cancers Team (EPICEA), Paris, France.
Abstract
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.
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.
Authors: Daniel B Spielman; Andi Liebowitz; Maeher Grewal; Chetan Safi; Jonathan B Overdevest; Alfred M Iloreta; Brett E Youngerman; David A Gudis Journal: World J Otorhinolaryngol Head Neck Surg Date: 2022-03-22