Esthesioneuroblastoma as an Unusual Cause for Dystopia

Abstract

Esthesioneuroblastoma, also known as olfactory neuroblastoma, is an uncommon malignant neoplasm arising from the olfactory epithelium in the roof of the nasal cavity. There are very few case reports published worldwide. The common presenting symptoms of Esthesioneuroblastoma are unilateral nasal obstruction (70%), epistaxis (50%), anosmia, rhinorrhoea, facial pain, headache, excessive lacrimation and rarely proptosis and visual disturbance. Apart from being locally aggressive, it metastasizes by haematogenous and lymphatic routes. We report an extremely rare case of esthesioneuroblastoma in a 20-year-old man with orbital involvement presenting as dystopia. This rare tumour should be considered in the differential diagnosis for young patients presenting to ophthalmic outpatient department with dystopia.

An isolated orbital mass with proptosis and diplopia: an uncommon metastasis from intracranial olfactory neuroblastoma in a Chinese patient

“…The location and morphology of the orbital tumor in our case was unique compared to the ones mentioned in previous studies. [4][5][6][7][8][9] Cytopathological and immunohistochemical features of the orbital neoplasm were found to be consistent with the features of primary intracranial tumors. So we speculate that the orbital neoplasm in our case is a metastasis rather than an infiltration from the primary lesion.…”

“…2,3 Cases of ONB with ophthalmologic presentations, including epiphora, decreased visual acuity, and proptosis, have been reported previously. [4][5][6][7][8][9] In these orbital malignant cases, the infiltrating neoplasm invaded orbits directly through bone destruction or the cranio-orbital communication anatomical structure. Herein we report a novel case of orbital metastasis from intracranial ONB in a Chinese patient demonstrating a possible uncommon approach of ONB migration to orbits.…”

“…[4][5][6][7][8][9] In orbital tumor cases, the infiltrating neoplasm invades the orbit by bone destruction, which can be clearly observed by imaging examinations. 12 However, in our patient's CT images, the orbital neoplasm was an isolated homogenous mass occupying the upper inner position of the right orbit, which does not support the possibility of direct invasion (Figure 1).…”