Multi-institutional Experience with Pediatric Olfactory Neuroblastoma

Background: Olfactory neuroblastoma is a rare malignancy arising from the olfactory epithelium. In adults, surgical resection followed by adjuvant radiation therapy is considered the standard of care. However, there is no established standard of care for pediatric olfactory neuroblastoma.

Objectives: The aim of the study is to review the current literature on pediatric olfactory neuroblastoma and report on the combined experience of treating this disease entity at three different institutions.

Methods: A retrospective multi-institutional review of patients diagnosed with olfactory neuroblastoma between 1995 and 2018 with an age at diagnosis less than 21 years old.

Results: Fifteen patients were identified with a mean age at diagnosis of 14.2 years old. Nine of the patients had dual modality treatment, while five had triple modality therapy. Of the patients treated with dual modality therapy, seven had surgery plus either chemotherapy or radiation therapy, while two patients were treated with chemoradiation therapy. Average follow-up duration was approximately 31 months. Thirteen patients were alive at last follow-up, 10 of whom were with no evidence of disease. Review of the medical literature suggests that pediatric olfactory neuroblastoma may be more advanced at presentation and responds well to neoadjuvant chemotherapy.

Conclusion: Our findings suggest that multimodality therapy is effective in the treatment of pediatric olfactory neuroblastoma. These results are encouraging, but further investigation in needed to determine the optimum treatment strategy.