Esthesioneuroblastoma: A case with prolonged disease control

Esthesioneuroblastoma, a rare tumor arising from the olfactory vault, varies from being indolent to extremely aggressive. Owing to its rarity, the diagnosis, staging, and treatment of the disease are not well defined. According to a number of small observational retrospective studies and case reports, the disease’s actual treatment involves surgery, radiotherapy, and/or chemotherapy (either as a single treatment or used in combination), depending on the disease’s staging. Optimal treatment has not been standardized, particularly regarding the role of chemotherapy. We describe a case of advanced esthesioneuroblastoma with prolonged disease control, subjected to a multimodal therapy with surgery, radiotherapy, and chemotherapy, illustrating the benefits of this approach in managing a patient with esthesioneuroblastoma. Herein, we analyze the most important and controversial issues of this type of neoplasia.