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

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.

Management of Esthesioneuroblastoma: A Retrospective Study of 6 Cases and Literature Review

“…The main prognostic factor is the clinical stage at the time of diagnosis. Unfavorable prognostic is associated with higher Kadish stages, lymph node extension, and distant metastases [ 43 , 44 , 45 ]. In another study, orbital and/or intracranial extension represented a factor of a poor prognostic [ 46 ].…”

Exclusively endoscopic surgical resection of esthesioneuroblastoma: A systematic review

“…Although esthesioneuroblastoma is the most common malignancy of the naval cavity in the pediatric patient population, it is nevertheless extremely rare, with fewer than 100 cases reported in the literature to date 5 . Because of its rarity, there is no consensus on treatment guidelines in this population.…”

“…Intraoperative complications in this group included moderate hemorrhage in one patient 48 . Only one study reported any postoperative complications in pediatric patients 5 . However, this study included patients treated with an open craniofacial resection as well and did not separate complications by surgical approach.…”

“… 48 Only one study reported any postoperative complications in pediatric patients. 5 However, this study included patients treated with an open craniofacial resection as well and did not separate complications by surgical approach. Multiple studies did report reduced postoperative morbidity and/or complication rates with endoscopic approach with similar oncologic outcomes.…”

“…Multiple studies did report reduced postoperative morbidity and/or complication rates with endoscopic approach with similar oncologic outcomes. 5 , 10 , 26 , 48 …”