Esthesioneuroblastoma: a case report

Esthesioneuroblastoma (olfactory neuroblastoma) is an uncommon neuroectodermal tumor. Its biological activity ranges from indolent growth to local recurrence and rapid widespread metastasis. Treatment options consist of surgical resection followed by radiation therapy for primary lesions and the addition of chemotherapy for advanced, recurrent, or metastatic lesions. Patients often present with nasal obstruction, rhinorrhea, recurrent epistaxis, hyposmia, or anosmia. We report a case of esthesioneuroblastoma involving bilateral nasal cavity leading to bilateral nasal obstruction, epistaxis and proptosis of the right eye associated with decreased visual acquity on that eye and loss of smell. A diffuse nontender, 6x6 cms swelling with illdefined margins was seen over the nasal bridge, extending superiorly to glabella and laterally to right maxillary region. X-ray PNS showed soft tissue mass in the nasal cavity with destruction of nasal septum, intense periosteal reaction with destruction of right maxillary wall and extension to right orbit. CT scan of paranasal sinuses showed 8.5 x 4.9 x 7.8 cms irregularly marginated heterogeneous iso- to hyper dense soft tissue mass lesion with extensive adjacent bony destruction and spiculated periosteal reaction involving bilateral nasal cavity and anterior cranial fossa. Biopsy from right nasal mass showed neuroblastoma. The patient received radiotherapy and chemotherapy. The modified Kadish staging system, lymph node status, treatment modality, and age are useful predictors of survival in patients who present with esthesioneuroblastoma. Excellent outcomes for esthesioneuroblastoma are achievable. Long-term follow-up is necessary because of the extended interval for recurrent disease; unlike most sinonasal malignancies, surgical salvage is possible.