Advanced Esthesioneuroblastoma Management: 20 Years Single-Center Experience

Introduction: Esthesioneuroblastoma is an uncommon tumor derived from the olfactory neuroepithelium that has a propensity for cranial base involvement through bone erosion and tumor extension to the anterior cranial fossa. It was first described in 1924 and has only around one thousand cases reported in the literature, which compromise the evaluation of the optimal treatment management.

Objectives: Evaluate the factors leading to the choice of treatment options and their impact in the outcome of survival and recurrence of disease. The demographic characteristics and disease staging at the time of diagnosis of the patients was registered and analyzed to look for risk factors and predictors of worse outcome.

Methods: We retrospectively reviewed the medical charts of patients with a confirmed anatomopathological diagnosis in the hospital database. The outcomes analyzed were overall survival and recurrence with comparison between different treatment groups: surgery alone; radiotherapy alone; radiochemotherapy alone; surgery followed by radiotherapy; surgery followed by radiochemotherapy; chemotherapy followed by surgery and radiotherapy.

Results: Twelve patients were identified in our database, the median age of diagnosis was 43 years old, ranging from 22 to 86 years old. Eight patients (66.6%) were male. Most patients presented with local nasal and sinus symptoms, as epistaxis and sinusitis; and only three patients (27%) had symptoms associated with central nervous system invasion (headaches and drowsiness), even though all patients had an advanced C Kadish stage, with anterior cranial fossa invasion. Two patients were lost to follow-up. From the analysis of the remaining ten patients, eight patients (80%) were alive with disease and two patients (20%) were dead of disease. The groups were divided in: 2 for surgery alone, 3 for surgery followed by radiotherapy, 1 for surgery followed by radiochemotherapy, 1 for chemotherapy followed by surgery and radiotherapy, 2 for radiotherapy alone and 2 for radiochemotherapy alone.

Conclusion: The rarity of esthesioneuroblastoma contributes to the lack of sufficient evidence for its management, which increases the importance of retrospective institutional reviews, showing the experience of a high-volume university neurosurgical service and expanding the body of published reports. We also have plans of adding our reports to that of another Brazilian service.

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