Esthesioneuroblastoma (ENB): A case report

Abstract

15546 Background: ENB is a rare embrionary tumor derived from neuroblasts of the olfactory sensory system. Polypoid mass with epistaxis or nasal obstruction are the most common clinical manifestations of this tumor. It’s invasive and it frequently causes regional and distant metastasis. ENB requires a multimodality therapy. The objective of this study is to describe the form of presentation, diagnosis, treatment and evolution of this tumor on a female patient (pt), as a casuistic contribution. Methods and Case Report: A 61 year old woman with a four month history of epistaxis, nasal pain and anosmia. CT shows nasal mass invading the entire nasal cavity and upper maxillary. Pt underwent nasal resection and reconstruction with frontal flaps. Anatomopathology: ENB invading the bone. Cromogranin (+), sinaptofisin (+), CK (−), NSE (−). Three months later: lesion on the right wing nasal and a mass in the soft palate. MRI: mass on the floor of the nasal fossa that involve the left maxillary sinus and the bone palate. Kadish stage C. We treated her with three cycles of chemotherapy using cisplatin 30 mg/sqM d 1–3 iv and etoposide 100 mg/sqM d 1–3 iv. After that, remission was observed in the wing nasal lesion but the the soft palate mass shows progression. MRI: mass in nasal fossa that destroys the left maxillary sinus and causes lysis of the upper maxillary and orbital floor. Pt was treated with radiotherapy (6000 cGy) showing complete remission of the nasal lession and partial response on the soft palate, verified by physical examination and RMI. Four months after the end of radiotherapy, she continues under control and maintains the response to therapy without evidence of progression. Conclusion: ENB is an unfrequent tumor without any standard treatment. In our case, the combination of surgery, CH and RT has been effective for local control of the disease with good tolerance and acceptable quality of life. No significant financial relationships to disclose.