AGGRESSIVE SALVAGE THERAPY OF OLFACTORY NEUROBLASTOMA CASE REPORT EXPERIENCE

Abstract

Objective

Olfactory neuroblastoma (ONB) is a rare malignant neoplasm arising from the olfactory neuroepithelium. It accounts for 3–5% of all nasal and Sinonasal malignancies, with an incidence of approximately 0.4 cases per million. A complete surgical resection of tumor followed by a full course of radiotherapy, is considered the treatment modality of choice for most ONBs. We aim to assess the impact of aggressive salvage radiotherapy in olfactory neuroblastoma on local recurrence and overall survival.

Case report

A 41-year-old Libyan female presented in 2020 with a mass in the right nasal cavity that caused persistent nasal congestion with intermittent epistaxis over one year ago. Histopathological characteristics and immunohistochemical findings of the biopsy confirmed an olfactory neuroblastoma grade III, Radiological imaging evaluation revealed group B stage, and an incomplete excision was done, followed by radical radiotherapy with 70 GY in 35 fractions over 5 weeks to the residual disease.

Methodology: Imaging follow-up for three years up to February 2024 shows no signs of local recurrence or distant metastasis.

Results

Although multi-disciplinary care is required, surgical treatment alone is effective for low-grade tumors with free margins. Adjuvant radiation is used for low-grade tumors with close margins, residual disease, or recurrent disease, and for all high-grade cancers. The poor prognosis associated with high-grade tumors may also mandate the addition of chemotherapy. Because recurrence can occur after 5 or even 10 years, aggressive management and long-term follow-up are mandatory.

Conclusion

Multimodal therapy, including post-operative radiotherapy of high-grade incompletely resected ONB, with precise treatment planning based on CT simulation, could achieve an excellent local control rate with acceptable toxicity and reasonable overall survival for patients with ONB. Still, the rarity of the disease makes it difficult to draw definitive conclusions about the role of systemic treatment in induction and concomitant settings.