Successful treatment of a patient with unresectable olfactory neuroblastoma: A case report and literature review.

Abstract

5620 Background: Olfactory Neuroblastoma (ONB), also known as esthesioneuroblastoma, is a rare neoplasm arising from the olfactory epithelium that commonly invades the orbit, paranasal sinuses and parapharyngeal space. Historically, surgery alone or surgery followed by radiation, have been the treatments of choice. Optimal management of ONB remains to be defined. We present the case of an unresectable Kadish stage C ONB treated with concomitant chemo-radiotherapy. Methods & Case Report: Our patient is a 59 year-old white female who presented with nasal stuffiness and increased lacrimation from the left eye. She was treated with antibiotics for several months but the swelling never improved. MRI showed a 6.4 x 3.7 cm left nasal mass with extensive involvement of the nasopharynx, sphenoid sinus, cavernous sinus, and the muscles of the left orbit. We treated her with radiation using 180 cGy per fraction of 4 MeV photons for a total of 7020 cGy. Concomitant 5-FU (750mg/sqM) was given on days 1 through 4 as a continuous infusion along with cisplatin (75mg/sqM) given over 48hrs on days 2 and 3. After 2 cycles of chemo-radiotherapy, the swelling of her left eye disappeared and she had a complete response verified by physical exam and MRI. We treated her with four cycles of adjuvant chemotherapy using cisplatin (100mg/sqM) on day 2 and etoposide (100mg/sqM) on days 1 through 3. RESULTS/DISCUSSION Neoadjuvant chemotherapy and radiotherapy has gained attention in recent years but the experience is under reported and fewer than 350 cases have been published worldwide since 1924 (Neurosurgery 2000;11:693). Responses to 5-fluorouracil, VP-16, adriamycin, cyclophosphomide, methotrexate, and vincristine have been attained, but the best results were obtained using cisplatin. CONCLUSIONS This is the first case report of unresectable ONB showing a complete response to concomitant chemo-radiotherapy and we plan to monitor the progression of her disease-free status over time. Much more research needs to be done and we hope that this would serve as an impetus for further study of unresectable ONB using concomitant chemo-radiotherapy. No significant financial relationships to disclose.