Esthesioneuroblastoma presenting as an orbital mass in a young child

Abstract

A 3-year-old boy presented with uniocular proptosis and ophthalmoplegia. Investigation revealed a mass involving the right orbit with extension into the left orbit and paranasal sinuses, and intracranial extension involving both frontal lobes. Biopsy of an enlarged cervical node and the intranasal mass revealed esthesioneuroblastoma. This tumor has been reported rarely in a child this age, and only 12 case reports document patients under 10 years of age. The presentation as an orbital mass is previously unreported and must now be considered in the differential diagnosis of proptosis in childhood. The usual clinical, radiological, and pathological features of olfactory esthesioneuroblastoma are reviewed.

Esthesioneuroblastoma in Childhood and Adolescence

“…ENB can occur in all ages, with peaks in the 2nd and 6th decades of life [1,5]. Generally, ENB is relatively rare in children [4,14,27,31]. The incidence in children up to 15 years is < 0.1 per 100,000 children (personal communication Dr. P. Kaatsch, German Childhood Cancer Registry, University of Mainz, Germany).…”

“…The incidence in children up to 15 years is < 0.1 per 100,000 children (personal communication Dr. P. Kaatsch, German Childhood Cancer Registry, University of Mainz, Germany). Bobele et al reported that only 20% of all ENB are diagnosed up to 20 years of age [4].…”

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).…”