2025 Proffered Presentations
S156: THE USE OF INDUCTION CHEMOTHERAPY IN OLFACTORY NEUROBLASTOMA: A MULTI-INSTITUTIONAL SERIES
Anthony Tang1; Samuel Adida1; Joao Paulo Almeida2; Pierre-Olivier Champagne3; Juan Fernandez-Miranda4; Paul Gardner5; Peter Hwang6; Jayaker Nayak6; Chirag Patel7; Zara Patel6; Maria Peris-Celda8; Carlos Pinheiro-Neto9; Olabisi Sanusi10; Carl Snyderman11; Brian D Thorp12; Jamie J Van Gompel8; Georgios A Zenonos5; Nathan T Zwagerman13; Eric W Wang11; Mathew Geltzeiler14; Garret W Choby11; 1University of Pittsburgh, School of Medicine; 2Department of Neurosurgery, Mayo Clinic, Jacksonville, Florida, USA; 3Department of Neurosurgery, CHU de Québec-Université Laval, Quebec City, Quebec, Canada.; 4Department of Neurosurgery, Stanford Hospital, Stanford, California.; 5Department of Neurological Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.; 6Department of Otolaryngology-Head and Neck Surgery, Stanford University School of Medicine, Palo Alto, California, USA; 7Department of Otolaryngology-Head and Neck Surgery, Loyola University Medical Center, Maywood, Illinois, United States; 8Department of Neurologic Surgery, Mayo Clinic,Rochester, MN; 9Department of Otorhinolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, US; 10Department of Neurosurgery, Oregon Health & Science University, Portland, Oregon, USA; 11Department of Otolaryngology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA; 12Department of Otolaryngology/Head and Neck Surgery at the University of North Carolina, Chapel Hill, North Carolina, USA; 13Department of Neurosurgery, Froedtert and the Medical College of Wisconsin, Milwaukee, Wisconsin, USA; 14Department of Otolaryngology-Head and Neck Surgery, Oregon Health & Science University, Portland, Oregon, USA
Introduction: Olfactory neuroblastoma (ONB) is a rare neuroectodermal tumor thought to originate from olfactory receptor cells of the nasal cavity. Negative-margin surgical resection may not be achievable in a subset of patients with locally advanced disease. Previous studies have shown that ONB is chemosensitive; further investigation is needed to explore the efficacy of induction chemotherapy (IC) to reduce tumor size and burden prior to definitive surgery. We present a modern-era multicenter ONB series of patients with locally advanced disease treated with IC.
Methods: A modern-era multi-center retrospective review of patients with ONB at 9 academic, tertiary-care centers within North America from 2005 to 2021 was performed. Only patients that were treated with induction chemotherapy were included in the study. Clinicopathologic features including histopathologic and imaging status, modified Kadish staging and Hyams grading systems, treatment modalities, margin-status, follow-up time, and survival were collected for analysis.
Results: Fifteen patients with ONB were treated with induction chemotherapy. The patient population was predominantly male (60%) with a median age of 51 (IQR, 40.5-64) years. Regarding baseline tumor characteristics in patients with available MRI, 72.7% patients had dural invasion, 16.7% had cavernous sinus invasion, 30.8% had periorbital involvement, and 16.7% had intraconal orbital tissue involvement. Of the 14 patients with modified Kadish staging, 7 (50%) patients were stage C and 7 (50%) patients were stage D prior to treatment. Two (22.2%) patients were Hyams grade I, 3 (33.3%) patients were Hyams grade II, 1 (11.1%) patient was Hyams grade III, and 3 (33.3%) patients were Hyams grade IV. Two (13.3%) patients were treated with induction chemotherapy followed by chemo/radiotherapy and 13 (86.7%) patients with induction chemotherapy followed by resection and adjuvant chemo/radiotherapy. Nine patients had pathological data available following resection, including 5 (55.6%) patients with negative margins and 4 (44.4%) patients with positive margins. Of the 4 patients with positive margins, all 4 had adjuvant RT with intensity-modulated radiation therapy. The median follow-up period of the cohort was 46 (IQR, 19.1-130.6) months after diagnosis. Of 14 patients with sufficient data for analysis, 3 (21.4%) patients developed local disease recurrence after a median of 52.0 (IQR, 29.2-99.2) months. Three patients developed regional disease recurrence after 59.7 (IQR, 39-93.95) months. One patient with modified Kadish stage D ONB had both local and distant recurrence at 6.5 months. Overall, 6 patients experienced recurrence after a median of 52 (IQR, 29.2-99.2) months. Of the 6 patients that had recurrence after IC, two patients had prior neck radiation before recurrence. Both of these patients had local recurrence only. The 5-year recurrence-free survival and overall survival were 59.7% and 70.6%, respectively.
Conclusion: Patients with ONB treated with IC tended to have more advanced disease at the time of treatment. Advanced-stage ONB tends to have lower rates of recurrence-free survival and overall survival. IC provides an additional treatment modality that has the potential to improve prognosis for patients with advanced-stage disease. Future large multi-institutional matched cohort studies are needed to delineate the role of IC in the management of this rare disease.