Orbital invasion by esthesioneuroblastoma

Abstract

Purpose: Esthesioneuroblastoma is a rare malignancy arising from the olfactory mucosa in the nasal fossa, which can invade the orbit producing ophthalmic symptoms and signs. The purpose of this study was to present a case series and review the literature to assess the characteristics of orbital involvement by esthesioneuroblastoma.

Methods: Retrospective case series of all patients treated for biopsy-proven esthesioneuroblastoma at the Royal Adelaide Hospital between 1992 and 2004.

Results: Nine patients (5 male, 4 female) with a mean age of 50 years (range, 20 to 66 years) were reviewed. One case was classified as Kadish stage A, 1 as Kadish stage B, and 7 as Kadish stage C. Mean time from onset of symptoms to diagnosis was 17 months (range, 2 to 24 months). Radiologic orbital invasion was present in 4 cases and was associated with ophthalmic symptoms or signs in 3 of these cases. These included proptosis (2 cases), periorbital pain (1 case), decreased visual acuity (1 case), extraocular muscle restriction (1 case), and chemosis (1 case). One patient with symptoms secondary to orbital invasion was initially referred to and assessed by an ophthalmologist. All patients who had or went on to have development of orbital invasion had advanced disease at diagnosis. Treatment was surgical (9/9), with the addition of radiotherapy (6/9) and chemotherapy (2/9). Mean follow-up was 3.6 years (range, 0.5 to 8.5 years). Six of 9 patients had tumor recurrence. Two had metastasis. Five of 9 patients remained alive.

Conclusions: Orbital invasion by esthesioneuroblastoma is not uncommon. It is important to be aware of this malignancy because a significant proportion of patients will present with ophthalmic signs and symptoms.