Abstract
Background: Esthesioneuroblastoma (ENB) is a rare sinonasal malignancy, lacking a unified staging system and treatment. Management at a single center was retrospectively evaluated to inform future treatment options and prognostic factors.Methods: Clinical data of 64 consecutive ENB patients, including prognostic factors and treatment methods, were reviewed retrospectively. Data were collected to calculate overall survival (OS) and progression free survival (PFS).Results: The majority of tumors 84.4% were within Kadish C stage, 79.7% were within T3 or T4, and 64.0% were within Hyams grade III or IV. A total of 50 (78.1%) patients received surgery and combined radiotherapy with or without chemotherapy, 10 (15.6%) received surgery with or without chemotherapy alone, and 4 (6.3%) received radiotherapy with or without chemotherapy alone. The majority of patients (79.7%) underwent endoscopic resection (endoscopic and endoscopically assisted). Surgery combined with radiotherapy with or without chemotherapy resulted in significantly better OS (84.4 vs. 50.6%, 84.4 vs. 37.5%) compared to surgery alone and radiotherapy alone (P = 0.0064). Endoscopic surgery group (endoscopic and endoscopically assisted) resulted in significantly better 5-year PFS (61.7 vs. 22.2%) compared to the open surgery group (P < 0.001). Although endoscopic surgery group was not a statistically significant predictor of 5-year OS (P = 0.54), the 5-year OS was 79.3% for the endoscopic surgery group and 76.2% for the open surgery group. A Cox regression analysis identified intracranial extension and surgery combined with radiotherapy as independent factors affecting 5-year OS while cervical lymph node metastasis and Hyams grade IV as independent factors affecting 5-year PFS.Conclusion: Our findings suggest that surgery combined with radiotherapy is the best treatment approach for ENB. For advanced tumors, endoscopic surgery is an effective treatment, and its survival rate is equal to or better than open surgery.
Highlights
Esthesioneuroblastoma (ENB), called olfactory neuroblastoma, is a primitive neuroectodermal tumor which is believed to originate from the olfactory epithelium of the upper nasal cavity
ENB is characterized by a prolonged natural history [1] and varying biological activity, ranging from a less invasive tumor leading to long-term survival, to a highly invasive neoplasm leading to rapid extensive metastasis with limited survival
Surgery combined with radiotherapy with or without chemotherapy resulted in significantly better overall survival (OS) (84.4 vs. 50.6%, 84.4 vs. 37.5%) compared to surgery alone and radiotherapy alone
Summary
Esthesioneuroblastoma (ENB), called olfactory neuroblastoma, is a primitive neuroectodermal tumor which is believed to originate from the olfactory epithelium of the upper nasal cavity. It was first described by Berger et al in 1924 and named “esthesioneuroepitheliome olfactif.”. The clinical manifestations of ENB are non-specific. ENB is misdiagnosed as other tumors, such as small cell carcinoma, melanoma, rhabdomyosarcoma, pituitary adenoma, sinonasal undifferentiated carcinoma, sinonasal neuroendocrine carcinoma, and lymphoma [2]. Pathology is the gold standard for the diagnosis of ENB. The pathological features of ENB include uniform small round cells, eosinophilic fiber background, true rosette (Flexner-Wintersteiner rosettes), or pseudorosette (Homer Wright rosettes) formations. Esthesioneuroblastoma (ENB) is a rare sinonasal malignancy, lacking a unified staging system and treatment. Management at a single center was retrospectively evaluated to inform future treatment options and prognostic factors
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