Approaches to the Treatment of Children with Esthesioneuroblastoma: Literature Review
Open Access
- 24 July 2019
- journal article
- Published by Paediatrician Publishers LLC in Oncopediatrics
- Vol. 6 (2), 78-86
- https://doi.org/10.15690/onco.v6i2.2019
Abstract
The incidence of esthesioneuroblastoma in children under 15 years of age is 0.1 per 100.000 children. Distinctive histological features of this tumor are diffuse accumulation of neuron-specific enolase, synaptophysin, chromogranin, and variable expression of cytokeratins. Diagnosis of the tumor includes endoscopic examination of the nasal cavity and nasopharynx, magnetic resonance imaging (MRI) and computed tomography (CT) of the skull base, paranasal sinuses with intravenous contrast. PET-CT is advisable to use for the detection of regional and distant metastases, as well as for suspected relapse. In patients of adult age, a negative effect on the outcome of the disease was detected, the detection of metastases in the lymph nodes of the neck, the presence of tumor cells at the edges of tumor resection and a high degree of malignancy of the tumor according to the Hyams system. Therapeutic approaches depend on the stage of esthesioneuroblastoma by Kadish. In the A-stage, surgical treatment is advisable. In the presence of tumor cells at the edges of the resection or residual tumor, radiation therapy is performed. In case of B-stage, surgical treatment is combined with the mandatory irradiation of the primary tumor area. In patients with the C-stage, neoadjuvant chemotherapy or radiation is performed, followed by a surgical treatment, adjuvant chemotherapy and/or radiation therapy. Patients with D-stage chemoradiation therapy is indicated. There is no consensus on an effective drug regimen. Overall 5-year survival varies significantly depending on the design of the study — 55% to 98%. Further study of the features of the clinical picture, morphological and molecular features and the course of the disease will help to improve our understanding of the nature of the tumor.Keywords
This publication has 31 references indexed in Scilit:
- DNA methylation-based reclassification of olfactory neuroblastomaActa Neuropathologica, 2018
- TRANSNASAL ENDOSCOPIC SURGERY IN COMPLEX TREATMENT OF ESTHESIONEUROBLASTOMA IN CHILDRENOncopediatrics, 2017
- Genetic and molecular alterations in olfactory neuroblastoma: implications for pathogenesis, prognosis and treatmentOncotarget, 2016
- Multimodality Treatment of Pediatric EsthesioneuroblastomaPediatric Blood & Cancer, 2015
- Esthesioneuroblastoma, Neuroendocrine Carcinoma, and Sinonasal Undifferentiated Carcinoma: Differentiation in Diagnosis and TreatmentInternational Archives of Otorhinolaryngology, 2014
- Long-Term Outcome of Esthesioneuroblastoma: Hyams Grade Predicts Patient SurvivalJournal of Neurological Surgery Part B: Skull Base, 2012
- Endoscopic versus open craniofacial resection of esthesioneuroblastoma: What is the evidence?The Laryngoscope, 2011
- Cancer of the Nasal Cavity in the Pediatric PopulationPEDIATRICS, 2008
- Langzeit-Erfahrungen in der Therapie des ÄsthesioneuroblastomsLaryngo-Rhino-Otologie, 2006
- Absence of EWSFL11 fusion in olfactory neuroblastomas indicates these tumors do not belong to the Ewing's sarcoma familyHuman Pathology, 1999