Esthesioneuroblastoma (olfactory neuroblastoma) with hemorrhage: an unusual presentation.

ABSTRACT

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. However, we report the highly unusual case of a patient with an esthesioneuroblastoma who presented with atypical symptoms of headaches, sinus congestion, and fatigue before acutely losing consciousness. Imaging showed a large frontal skull-based tumor associated with intratumoral hemorrhage. The findings prompted an emergent combined anterior craniofacial resection with gross total resection of the tumor. Except for anosmia, the patient recovered almost completely. Postoperatively, she received adjuvant intensity-modulated radiation therapy and chemotherapy. This is the first reported case of an esthesioneuroblastoma presenting with hemorrhage and rapidly declining mental status, an acute neurological manifestation of which clinicians should be aware.