Esthesioneuroblastoma Presenting as a Large Cervicofacial Mass: A Case Report and Review of the Literature

Abstract

Ichraq Horrane*, Zineb Berdi, Moad El Mekkaoui, Zakaria El Hafi, Zakaria Arkoubi, Razika Bencheikh, Anas Benbouzid and Leila Essakalli

Introduction and Importance: Esthesioneuroblastoma (ENB) is a rare malignant tumor arising from the olfactory neuroepithelium, accounting for a small proportion of sinonasal cancers. Its clinical presentation is often nonspecific, and advanced disease may manifest with orbital, intracranial, or cervical involvement, posing significant diagnostic and therapeutic challenges.

Case Presentation: We report the case of a 31-year-old man presenting with progressive nasal obstruction, epistaxis, anosmia, and headaches, followed by the rapid development of a large cervicofacial mass. Imaging revealed an aggressive sinonasal tumor with orbital, intracranial, and cervical lymph node involvement. Histopathological and immunohistochemical analysis confirmed a low-grade esthesioneuroblastoma (Hyams I–II), classified as Kadish stage C. Due to unresectability, the patient was treated with combined chemoradiotherapy.

Clinical Discussion: Esthesioneuroblastoma is characterized by heterogeneous clinical behavior and a high propensity for local extension and recurrence. Advanced-stage disease often requires multimodal treatment. Imaging and histopathological grading are essential for staging, prognostic assessment, and therapeutic decision-making.

Conclusion: This case highlights an unusual cervicofacial presentation of advanced esthesioneuroblastoma and underscores the importance of early diagnosis, comprehensive imaging, and multidisciplinary management to improve outcomes in this rare malignancy.

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