{"entity":{"id":"sinonasal","kind":"cancer","name":"Nasal cavity and paranasal sinus cancers (including esthesioneuroblastoma)","aka":["Sinonasal cancer","Esthesioneuroblastoma","Olfactory neuroblastoma","Sinonasal undifferentiated carcinoma","SNUC","Paranasal sinus cancer"],"tldr":"Cancers of the nose and sinuses are a mixed group, from squamous carcinoma to the nerve-derived esthesioneuroblastoma and the aggressive undifferentiated carcinoma SNUC. Surgery through the nose with an endoscope followed by precise radiotherapy has replaced disfiguring open operations, and giving chemotherapy first to see who responds now guides how SNUC is treated.","summary":"Sinonasal malignancies arise in the nasal cavity, maxillary and ethmoid sinuses and comprise squamous cell carcinoma (the majority, including HPV-related and inverted-papilloma-associated forms), intestinal-type adenocarcinoma (strongly linked to occupational wood and leather dust), esthesioneuroblastoma (olfactory neuroblastoma, graded by Hyams), sinonasal undifferentiated carcinoma (SNUC), neuroendocrine carcinoma, adenoid cystic carcinoma, mucosal melanoma, NUT carcinoma and SMARCB1- or SMARCA4-deficient carcinomas. Molecular reclassification has split SNUC: about half carry IDH2 R172 mutations, and others are SWI/SNF-deficient; DEK-AFF2 fusion defines a distinct carcinoma. Proximity to orbit, skull base and brain dictates both symptoms (late presentation with obstruction, epistaxis, proptosis) and treatment complexity.\n\nSurgery is the mainstay for resectable tumours, and endoscopic endonasal and combined cranioendoscopic approaches have replaced craniofacial resection for most, with equivalent control and less morbidity. Post-operative IMRT or proton therapy is standard for advanced stage, close margins, high grade and esthesioneuroblastoma; protons reduce dose to optic pathways and brain. SNUC is treated with induction platinum-etoposide chemotherapy, and the response-adapted approach from MD Anderson (JAMA Oncol 2019) directs responders to definitive chemoradiotherapy and non-responders to surgery, with better outcomes than surgery-first. Esthesioneuroblastoma is managed by resection and radiotherapy, with chemotherapy for high Hyams grade or advanced disease.\n\nFrontiers are IDH2 inhibitors for IDH2-mutant SNUC (case reports and early trials with enasidenib), SWI/SNF-directed therapy, HPV-based prognostication, and multi-institutional consortia to run trials in these rare histologies.","asOf":"2026-09-10","wikipedia":"https://en.wikipedia.org/wiki/Esthesioneuroblastoma","links":[{"label":"NCI PDQ: paranasal sinus and nasal cavity cancer","url":"https://www.cancer.gov/types/head-and-neck/patient/adult/paranasal-sinus-treatment-pdq"},{"label":"NCCN Head and Neck Cancers","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1437"},{"label":"Response-adapted SNUC treatment (JAMA Oncol 2019)","url":"https://doi.org/10.1001/jamaoncol.2018.5526"}],"tags":["nci-coverage","rare","head-and-neck"],"related":[],"cancers":[],"sections":[],"technologies":["imrt-igrt","proton-therapy","robotic-surgery","cytotoxic-chemotherapy","checkpoint-inhibitor"],"targets":["idh","pd1"],"drugs":["cisplatin","etoposide","platinum-etoposide","pembrolizumab","nivolumab","enasidenib"],"companies":[],"institutions":[],"pathways":["idh-2hg","swi-snf-chromatin"],"terms":["head-neck-subsites","hpv-status","rare-cancers"],"trials":[],"people":[],"bottlenecks":["b-rare-cancers"],"keyPapers":["paper-chen-jama-oncol"],"journals":[],"dependsOn":[],"notes":[],"group":"head and neck","burden":"About 3 to 5 percent of head and neck cancers, or roughly one case per 100,000 per year; more common in men and in woodworkers, leather and nickel workers.","subtypes":["Sinonasal squamous cell carcinoma (keratinising, non-keratinising, HPV-related)","Intestinal-type adenocarcinoma (occupational)","Esthesioneuroblastoma (Hyams grade I to IV)","Sinonasal undifferentiated carcinoma (IDH2-mutant and other)","SMARCB1- and SMARCA4-deficient sinonasal carcinoma","Sinonasal mucosal melanoma","Adenoid cystic carcinoma of the sinonasal tract"],"biomarkers":["Histological subtype and Hyams grade (esthesioneuroblastoma)","IDH2 R172 mutation (SNUC)","SMARCB1/SMARCA4 loss","NUT immunohistochemistry to exclude NUT carcinoma","HPV status (non-keratinising squamous)","Occupational exposure history (wood dust)"],"standardOfCare":[{"setting":"Resectable squamous carcinoma, adenocarcinoma, esthesioneuroblastoma","approach":"Endoscopic or cranioendoscopic resection with negative margins followed by IMRT or proton therapy for advanced stage, high grade or close margins; concurrent cisplatin for positive margins or nodal disease.","refs":["imrt-igrt","proton-therapy","cisplatin","robotic-surgery"],"guideline":{"nccn":"Category 2A","version":"NCCN Head and Neck Cancers (ethmoid and maxillary sinus tumours)","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1437"}},{"setting":"Sinonasal undifferentiated carcinoma","approach":"Induction platinum-etoposide; responders proceed to definitive chemoradiotherapy, non-responders to surgery plus radiotherapy (response-adapted approach, JAMA Oncol 2019).","refs":["cisplatin","etoposide","platinum-etoposide","imrt-igrt"],"guideline":{"version":"Amit and colleagues, JAMA Oncol 2019","url":"https://doi.org/10.1001/jamaoncol.2018.5526"}},{"setting":"Unresectable or metastatic","approach":"Platinum-based chemotherapy, and pembrolizumab or nivolumab per head and neck squamous indications for squamous histology; trials for IDH2-mutant SNUC (enasidenib) and SWI/SNF-deficient carcinoma.","refs":["pembrolizumab","nivolumab","enasidenib"]}],"stateOfArt":["Endoscopic skull-base surgery has replaced open craniofacial resection for most tumours with equivalent oncological outcomes and far less morbidity.","Response to induction chemotherapy is now used as a biomarker in SNUC to decide between organ-preserving chemoradiotherapy and surgery.","Molecular splitting of SNUC into IDH2-mutant and SWI/SNF-deficient groups has given the first targetable alterations in a disease that had none.","Proton therapy reduces dose to optic nerves and brain in tumours that sit against both."],"history":[{"year":1924,"title":"Esthesioneuroblastoma first described (Berger and Luc)","refs":[]},{"year":1965,"title":"Wood dust linked to sinonasal adenocarcinoma","note":"Occupational epidemiology in the UK furniture industry (Acheson).","refs":[]},{"year":1986,"title":"Frierson describes sinonasal undifferentiated carcinoma","refs":[]},{"year":1988,"title":"Hyams grading of esthesioneuroblastoma","refs":[]},{"year":2000,"title":"Endoscopic endonasal resection of sinonasal cancer established","note":"Series from Pittsburgh and Europe show equivalence to open surgery.","refs":[]},{"year":2017,"title":"IDH2 mutations define a SNUC subset","note":"Dogan and colleagues; Jo and colleagues.","refs":[]},{"year":2019,"title":"Response-adapted treatment of SNUC","note":"Amit and colleagues, JAMA Oncol.","refs":["cisplatin","etoposide"]}],"pipeline":["proton-therapy","enasidenib","pembrolizumab"],"openProblems":["Late presentation: most tumours are locally advanced at diagnosis; awareness in ENT clinics and imaging AI are the response.","No randomised trials exist for any sinonasal histology; international consortia are pooling cases.","IDH2-mutant SNUC: prospective trials of IDH2 inhibitors.","Visual and neurocognitive toxicity of radiotherapy: protons and reduced-margin planning."]},"route":"/cancers/sinonasal/","neighbours":{"technology":[{"id":"cytotoxic-chemotherapy","kind":"technology","name":"Cytotoxic chemotherapy","route":"/technologies/cytotoxic-chemotherapy/"},{"id":"checkpoint-inhibitor","kind":"technology","name":"Immune checkpoint inhibitors","route":"/technologies/checkpoint-inhibitor/"},{"id":"imrt-igrt","kind":"technology","name":"IMRT / IGRT (modern external beam)","route":"/technologies/imrt-igrt/"},{"id":"proton-therapy","kind":"technology","name":"Proton therapy","route":"/technologies/proton-therapy/"},{"id":"robotic-surgery","kind":"technology","name":"Robotic & minimally invasive surgery","route":"/technologies/robotic-surgery/"}],"target":[{"id":"idh","kind":"target","name":"IDH1 / IDH2","route":"/targets/idh/"},{"id":"pd1","kind":"target","name":"PD-1","route":"/targets/pd1/"}],"drug":[{"id":"cisplatin","kind":"drug","name":"Cisplatin","route":"/drugs/cisplatin/"},{"id":"enasidenib","kind":"drug","name":"Enasidenib","route":"/drugs/enasidenib/"},{"id":"etoposide","kind":"drug","name":"Etoposide","route":"/drugs/etoposide/"},{"id":"nivolumab","kind":"drug","name":"Nivolumab","route":"/drugs/nivolumab/"},{"id":"pembrolizumab","kind":"drug","name":"Pembrolizumab","route":"/drugs/pembrolizumab/"},{"id":"platinum-etoposide","kind":"drug","name":"Platinum + etoposide (EP / CE)","route":"/drugs/platinum-etoposide/"}],"pathway":[{"id":"idh-2hg","kind":"pathway","name":"Mutant IDH / 2-hydroxyglutarate","route":"/pathways/idh-2hg/"},{"id":"swi-snf-chromatin","kind":"pathway","name":"SWI/SNF chromatin remodelling","route":"/pathways/swi-snf-chromatin/"}],"term":[{"id":"head-neck-subsites","kind":"term","name":"Head and neck subsites (oral cavity, oropharynx, larynx)","route":"/terms/head-neck-subsites/"},{"id":"hpv-status","kind":"term","name":"HPV status (HPV-positive / HPV-negative)","route":"/terms/hpv-status/"},{"id":"rare-cancers","kind":"term","name":"Rare cancers","route":"/terms/rare-cancers/"}],"bottleneck":[{"id":"b-rare-cancers","kind":"bottleneck","name":"Rare and paediatric cancers without markets","route":"/bottlenecks/b-rare-cancers/"}],"paper":[{"id":"paper-chen-jama-oncol","kind":"paper","name":"Anlotinib for Refractory Advanced Non-Small Cell Lung Cancer in China","route":"/key-papers/paper-chen-jama-oncol/"}],"trial":[{"id":"nct03274414","kind":"trial","name":"A Clinical Trial of Endoscopic Surgery Followed by Chemotherapy and Proton Radiation for the Treatment of Tumors in the Sinus and Nasal Passages","route":"/trials/nct03274414/"}],"cancer":[{"id":"esthesioneuroblastoma","kind":"cancer","name":"Esthesioneuroblastoma (olfactory neuroblastoma)","route":"/cancers/esthesioneuroblastoma/"},{"id":"rare-childhood-cancers","kind":"cancer","name":"Rare cancers of childhood (NCI PDQ umbrella)","route":"/cancers/rare-childhood-cancers/"},{"id":"sinonasal-undifferentiated-carcinoma","kind":"cancer","name":"Sinonasal undifferentiated carcinoma (SNUC) and SWI/SNF-deficient sinonasal carcinoma","route":"/cancers/sinonasal-undifferentiated-carcinoma/"}]}}