Kevin Wu1, Sarah A Avila1, Rupak Bhuyan1, Ammar Matloob2, Anthony G Del Signore3, Constantinos Hadjipanayis4, James Chelnis1. 1. Department of Ophthalmology, Icahn School of Medicine at Mount Sinai, New York, NY, USA. 2. Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, USA. 3. Department of Otolaryngology, Mount Sinai Beth Israel, New York, NY, USA. 4. Department of Neurosurgery, Mount Sinai Beth Israel, New York, NY, USA.
Abstract
PURPOSE: To review the current literature on esthesioneuroblastoma (ENB) as it pertains to clinical features, grading systems, treatment options, and survival. METHODS: A literature search in PubMed was performed to include all articles published in English with orbit involving ENB. Only articles that included each patient's demographics, tumor stage, treatment, or survival were included. A total of 22 articles with 104 patients were considered for this literature review. We also present five cases of ENB, all encountered in our health system, between 2010 and 2020. RESULTS: The median age of diagnosis of orbit involving ENB was 44.5 years. Males were more likely affected than females at 72.9%. Common presenting ocular symptoms were visual change (38.1%), periorbital pain (33.3%), and diplopia (14.3%). Common clinical exam findings were proptosis (47.6%), extraocular movement deficit (23.8%), and periorbital edema (19.0%). Twenty-seven patients (77.1%) received surgery, 22 patients (62.9%) received chemotherapy, and 30 patients (85.7%) received radiation therapy as part of their treatment. Median duration of survival was 124.0 months and 5-year overall survival (OS) was 67.1%. Hyams, Kadish, and Dulguerov T-staging showed inconsistent survival prognosis while orbital invasion and lymph node metastasis had worse outcomes. Our five cases exhibited the spectrum of disease processes evidenced above, with four involving the orbit. CONCLUSIONS: ENB is a rare sinonasal tumor that can invade the orbit. Because of its rarity, no single staging system appears superior. Resection with radiation therapy has superior survival results while the benefits of chemotherapy are currently unknown.
PURPOSE: To review the current literature on esthesioneuroblastoma (ENB) as it pertains to clinical features, grading systems, treatment options, and survival. METHODS: A literature search in PubMed was performed to include all articles published in English with orbit involving ENB. Only articles that included each patient's demographics, tumor stage, treatment, or survival were included. A total of 22 articles with 104 patients were considered for this literature review. We also present five cases of ENB, all encountered in our health system, between 2010 and 2020. RESULTS: The median age of diagnosis of orbit involving ENB was 44.5 years. Males were more likely affected than females at 72.9%. Common presenting ocular symptoms were visual change (38.1%), periorbital pain (33.3%), and diplopia (14.3%). Common clinical exam findings were proptosis (47.6%), extraocular movement deficit (23.8%), and periorbital edema (19.0%). Twenty-seven patients (77.1%) received surgery, 22 patients (62.9%) received chemotherapy, and 30 patients (85.7%) received radiation therapy as part of their treatment. Median duration of survival was 124.0 months and 5-year overall survival (OS) was 67.1%. Hyams, Kadish, and Dulguerov T-staging showed inconsistent survival prognosis while orbital invasion and lymph node metastasis had worse outcomes. Our five cases exhibited the spectrum of disease processes evidenced above, with four involving the orbit. CONCLUSIONS: ENB is a rare sinonasal tumor that can invade the orbit. Because of its rarity, no single staging system appears superior. Resection with radiation therapy has superior survival results while the benefits of chemotherapy are currently unknown.
Authors: Vithor Ely B da Silva; Samuel R Buniatti; Felipe D'Almeida Costa; Mauricio R Torrecillas; Jean G de Oliveira; Marcio S Rassi Journal: Front Surg Date: 2022-04-19