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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.
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.
Surgery 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.
Frontiers 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.
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.
Site decides cause and behaviour: HPV drives oropharyngeal cancer, EBV drives nasopharyngeal cancer, tobacco drives oral and laryngeal cancer; all drain into the neck node levels that surgeons and radiotherapists map.
Same organ: Acinic cell carcinoma of the salivary glands, Carcinoma ex pleomorphic adenoma, Multiple endocrine neoplasia type 1 (MEN1), Multiple endocrine neoplasia type 2 (MEN2A and MEN2B), Hyperparathyroidism-jaw tumour syndrome (CDC73-related parathyroid carcinoma), Oropharyngeal cancer (tonsil and base of tongue), Laryngeal and hypopharyngeal cancer, Oral cavity cancer (mouth and tongue), Head and neck squamous cell carcinoma, Nasopharyngeal carcinoma, Salivary gland cancers, Papillary thyroid cancer, Follicular thyroid cancer, Medullary thyroid cancer, Anaplastic thyroid cancer, Thyroid cancer, NUT carcinoma (midline carcinoma with NUTM1 rearrangement), Parathyroid carcinoma, Multiple endocrine neoplasia syndromes (MEN1, MEN2, MEN4), HPV-positive oropharyngeal cancer, HPV-negative head and neck squamous cell carcinoma (including HPV-negative oropharyngeal cancer), Recurrent or metastatic head and neck squamous cell carcinoma, Hypopharyngeal cancer, Adenoid cystic carcinoma, Salivary duct carcinoma, Mucoepidermoid carcinoma, Oral tongue and floor of mouth cancer, Buccal mucosa and gingivobuccal cancer (oral cancer in India), Lip cancer, Locoregionally advanced nasopharyngeal carcinoma (stage III to IVA), Recurrent and metastatic nasopharyngeal carcinoma, Esthesioneuroblastoma (olfactory neuroblastoma), Sinonasal undifferentiated carcinoma (SNUC) and SWI/SNF-deficient sinonasal carcinoma
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.
Induction platinum-etoposide; responders proceed to definitive chemoradiotherapy, non-responders to surgery plus radiotherapy (response-adapted approach, JAMA Oncol 2019).
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.
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Query for this cancer: (TITLE:"Nasal cavity and paranasal sinus cancers" OR ABSTRACT:"Nasal cavity and paranasal sinus cancers" OR TITLE:"including esthesioneuroblastoma" OR ABSTRACT:"including esthesioneuroblastoma" OR TITLE:"Sinonasal cancer" OR ABSTRACT:"Sinonasal cancer" OR TITLE:"Esthesioneuroblastoma" OR ABSTRACT:"Esthesioneuroblastoma" OR TITLE:"Olfactory neuroblastoma" OR ABSTRACT:"Olfactory neuroblastoma" OR TITLE:"Sinonasal undifferentiated carcinoma" OR ABSTRACT:"Sinonasal undifferentiated carcinoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Nasal cavity and paranasal sinus cancers (including esthesioneuroblastoma), not a curated reading list.
Occupational epidemiology in the UK furniture industry (Acheson).
Series from Pittsburgh and Europe show equivalence to open surgery.
Dogan and colleagues; Jo and colleagues.
Amit and colleagues, JAMA Oncol.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
Fever, cough or breathlessness, rapid weight gain or swelling, bone pain, low blood pressure or reduced urine; the labels say to start steroids and monitor at the first suspicion, and the syndrome has been fatal.
No pharmacokinetic interactions expected (antibody). See the irAE guide for toxicity management.
Dose reduce or avoid for CrCl below 60 (carboplatin is the alternative).
Reduce to 75% for CrCl 15-50.
See all on the product pages:CisplatinEnasidenibEtoposideNivolumabPembrolizumabPlatinum + etoposide (EP / CE)·Printable cards in the navigator
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