Ectopic Primary Olfactory Neuroblastoma: Case Series and Literature Review

Olfactory neuroblastoma (ONB) is a rare malignant tumor arising in the upper nasal cleft. Rarely, ONB may originate in ectopic sites and the impact of this on prognosis and treatment strategies continues to be debated.

A retrospective analysis was undertaken of patients with ectopic ONB treated between 2000 and 2020 in a tertiary-care referral center for skull base tumors. Three patients were included in this analysis: a 37-year-old woman with ONB arising from the bulla ethmoidalis; a 28-year-old man with inappropriate secretion of antidiuretic hormone caused by a maxillary sinus ONB; and a 41-year-old man with lacrimal sac ONB. Preoperative workup, surgical approach, adjuvant treatments and postoperative surveillance were analyzed. Relevant literature published between 2000 and January 2021 was fully reviewed to investigate oncologic outcomes and delineate the standard of care for such rare tumors.

All patients were treated via endoscopic endonasal resection with radical intent, followed by adjuvant treatments when required. No recurrences of disease were observed after a mean follow-up time of 32 months (range, 12-60 months). Data emerging from the literature suggest that a multidisciplinary treatment approach, including free-margins surgical resection followed by adjuvant radiotherapy or radiochemotherapy, is recommended. Olfactory bulb and dura preservation should be attempted whenever feasible.

Endoscopic endonasal surgery should be preferred, when possible, to achieve complete excision to minimize patients' morbidity. The ectopic site of origin affects prognosis and should be considered when selecting the appropriate multimodal treatment strategy.