Evolution of treatment for children with esthesioneuroblastoma (1969–2019)

Abstract

Introduction. Esthesioneuroblastoma is a rare neuroectodermal malignant tumor. The incidence of this tumor in children under 15 years of age is 0.1 per 100,000 pediatric population. The information on incidence, disease course, and treatment of esthesioneuroblastoma in children vary significantly and depend on the follow-up period and statistical methods.Objective – to assess risk factors for esthesioneuroblastoma and evaluate treatment strategies used in children and their prospects.Materials and methods. This study included 29 patients aged 2 to 17 years diagnosed with esthesioneuroblastoma and treated at the Research Institute of Oncology and Hematology, N.N. Blokhin Russian Cancer Research Center, Ministry of Health of Russia between 1969 and 2019. The median age upon diagnosis was 10 years. Six patients (20 %) developed the disease early in their life (being under 3 years of age). Esthesioneuroblastoma was equally common in boys and girls. More than half of the patients (n = 15; 51 %) had stage IV disease. The primary tumor invaded the maxillary sinus (n = 25; 86 %), orbit (n = 10; 34 %), ethmoid sinus (n = 18; 62 %), and cranial cavity (n = 12; 41 %). The majority of children (n = 28; 96 %) received pharmacotherapy and radiotherapy (n = 27; 93 %); the total focal dose was 50 Gy in case of primary tumor. The affected cervical lymph nodes were irradiated in 10 (35 %) patients (total focal dose 40 Gy). Ten (35 %) patients have undergone surgery.Results. The follow-up period varied between 3 months and 50 years. Nine (34 %) patients are still alive. Fourteen patients (48 %) died due to tumor progression; 1 (3 %) patient died due to complications from special treatment. Five (17 %) patients were lost to follow-up at different stages.Conclusion. The following risk factors were associated with poor prognosis in children with esthesioneuroblastoma: disease onset in early childhood, no adequate diagnosis of distant metastases prior to specific treatment initiation, disseminated disease, and high Ki-67 index. In patients with advanced cancer, surgeries were effective only in combination with chemoradiotherapy. Long-term treatment outcomes in children with esthesioneuroblastoma are poor due to high risk of recurrence and tumor dissemination.