Pattern of loco‐regional relapses and treatment in pediatric esthesioneuroblastoma: The French very rare tumors group ( Fracture ) contribution - Université Paris Cité Accéder directement au contenu
Article Dans Une Revue Pediatric Blood and Cancer Année : 2020

Pattern of loco‐regional relapses and treatment in pediatric esthesioneuroblastoma: The French very rare tumors group ( Fracture ) contribution

Résumé

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.
Fichier non déposé

Dates et versions

hal-04031016 , version 1 (15-03-2023)

Identifiants

Citer

Benoît Dumont, Brice Fresneau, Line Claude, Anne‐sophie Defachelles, Vincent Couloigner, et al.. Pattern of loco‐regional relapses and treatment in pediatric esthesioneuroblastoma: The French very rare tumors group ( Fracture ) contribution. Pediatric Blood and Cancer, 2020, 67 (4), ⟨10.1002/pbc.28154⟩. ⟨hal-04031016⟩
14 Consultations
0 Téléchargements

Altmetric

Partager

Gmail Facebook X LinkedIn More