OLFACTORY NEUROBLASTOMA: A CASE SERIES FROM A SINGLE INSTITUTION

Abstract

<h3>Introduction</h3> Olfactory neuroblastoma (ONB) is an uncommon malignant neuroectodermal sinonasal tumor, which comprises approximately 2% to 3% of all sinonasal tumors. These tumors originate from specialized sensory neuroepithelial/neuroectodermal olfactory cells and occur most frequently in the upper nasal cavity, in the region of the cribriform plate. All age groups are affected, and patients often present with locally advanced disease resulting from delay in presentation. This is attributed to the slow growth by tumors and nonspecific symptomatology. <h3>Objective</h3> To evaluate the histologic features and management outcomes for patients with ONB at our institution. <h3>Results</h3> Over a 10-year period (2009-2019) 6 patients were identified with ONB. The majority of tumors (n = 4) were locally advanced at the time of presentation with extension beyond the nasal cavity and paranasal sinuses (Kadish stage C). One tumor was stage B, and one was stage A. Three tumors were Hyams grade I, 2 tumors were grade II, and 1 tumor was grade III. One grade I tumor demonstrated ganglioneuromatous differentiation. Glandular differentiation was seen in 1 grade II ONB. All patients had surgical resection, with 3 patients receiving postoperative radiotherapy. One patient was found to have multiple bony and left cervical metastases by cross-sectional staging imaging and therefore received postoperative chemotherapy. All patients are alive with 4 patients free from disease (follow-up period ranging from 1-108 months). <h3>Conclusions</h3> At our center patients typically presented with advanced disease with most patients receiving multimodality treatment comprising surgery and radiotherapy. All patients are alive with only 1 patient with disseminated disease.