Outcomes of Orbit-Sparing Treatment in Olfactory Neuroblastoma with Orbital Involvement: A Retrospective Cohort Study

Background: Olfactory neuroblastoma (ONB) is a rare and aggressive sinonasal malignancy with a propensity to invade local structures, including the orbit, which poses unique clinical challenges. Treatment decisions in such cases require careful consideration of orbit-sparing versus non-orbit-sparing approaches, a topic underrepresented in the existing literature. This retrospective cohort study aimed to evaluate both functional and oncologic outcomes in patients with ONB and orbital involvement.

Methods: We conducted a retrospective analysis of patients diagnosed with ONB at our institution between 1996 and 2019, focusing on those with documented orbital invasion on pre-operative imaging. We classified the cohort into four distinct groups based on the extent of orbital involvement: (1) lamina abutted but intact; (2) lamina thinned (partial thickness erosion), but globe not displaced; (3) lamina eroded, and globe displaced but periorbita not invaded; (4) orbit and periorbita grossly invaded. Extent of orbital involvement was determined using a combination of preoperative CT and/or MR imaging, and confirmed via findings reported in operative notes, and pathology reports if available. Alongside these classifications, we recorded demographic data, disease staging, tumor grading, presenting symptoms, treatment modalities, and functional and oncologic outcomes. Descriptive statistics, Fisher’s exact tests for categorical variables, and Kruskal–Wallis analysis for numerical variables were employed for data analysis.

Results: Our institutional ONB database included a total of 142 patients, of which 42 (30%) demonstrated orbital involvement on preoperative imaging. Excluding two patients who underwent orbital exenteration, the remaining 40 patients underwent surgical interventions with orbital preservation. The median age of the cohort was 53 years, with a predominantly male population (65%). The distribution among the four groups was as follows: group 1 = 13 (33%); group 2 = 14 (35%); group 3 = 4 (8%); group 4 = 10 (25%). Approximately 18% of patients presented with visual symptoms, which included proptosis, visual blurriness, diplopia, and periorbital edema. Gross total resection was achieved in 35 patients (88%), with group 4 patients more likely to undergo subtotal resection (p = 0.003). Margin-negative resection was achieved in 24 patients (60%). Sixteen patients (40%) experienced postoperative functional deficits, with five exhibiting immediate deficits (within 14 days following surgery) and 12 experiencing delayed deficits. Of note, five patients with deficits regained full visual function. The median follow-up period was 94.5 months. Regarding recurrence, at the most recent follow-up, 23 patients (57.5%) experienced disease recurrence. Notably, no correlation was observed between the extent of orbital invasion and the likelihood of recurrence at any site (orbital, local, regional, or distant). In terms of survival, at the most recent follow-up, 27 patients (67.5%) were alive, and 13 (32.5%) were deceased. There were no discernible differences in survival based on the degree of orbital invasion.

Conclusions: Orbital preservation can be achieved with minimal functional deficits and reasonable oncologic outcomes in most cases of ONB with orbital involvement. This emphasizes the potential advantages of orbit-sparing approaches in managing this complex disease.

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Artikel online veröffentlicht:
05. Februar 2024