A rare case of metastatic esthesioneuroblastoma

Abstract

Olfactory neuroblastoma, also known as esthesioneuroblastoma (ENB), is a rare malignant tumour of the basal layer of the olfactory epithelium, which originally develops unilaterally, accounting for 3-6% of all intranasal tumours. We present the case of a patient with a voluminous ethmoidal lesion that invaded the left basal frontal lobe and left orbit. The biopsy revealed a stage C KADISH, grade III neuroblastoma. The patient followed a multimodal treatment with chemotherapy and radiation therapy to which he responded partially, then returned after 11 months for sphincter disorder and bilateral sciatic type pain. An MRI showed metastasis of the filum terminale, the anatomopathological exam identifying also neuroblastoma. CT and MRI imaging are required for a correct assessment of the regional extension of olfactory neuroblastoma, response to oncological treatment but also for the detection of secondary lesions found in a small number of cases.