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Sinonasal undifferentiated carcinoma is a rare, fast-growing cancer of the nasal cavity and sinuses made of primitive cells with no clear line of differentiation, most carrying an IDH2 mutation. It presents as a large mass pressing on the eye or brain and is treated with chemotherapy first, then surgery or chemoradiotherapy depending on response; SMARCB1- or SMARCA4-deficient tumours are separate.
Sinonasal undifferentiated carcinoma was defined in 1986 as a high-grade carcinoma of the sinonasal tract without squamous or glandular differentiation, negative for EBV and for the neuroendocrine and NUT markers that define its mimics. Genomic studies from 2017 found IDH2 R172 mutations in most cases, giving the tumour a molecular identity and a possible drug target; the 2022 WHO classification also carved out SMARCB1 (INI1)-deficient and SMARCA4-deficient sinonasal carcinomas, which look similar but are driven by loss of SWI/SNF chromatin remodelling proteins, and NUT carcinoma, which has its own page. All present late with a bulky mass causing nasal obstruction, proptosis, diplopia or headache, invading the orbit, skull base and dura, and about a fifth have neck node metastases at diagnosis.
Historically, surgery followed by radiotherapy cured few patients because of the extent of disease, and the MD Anderson group changed the paradigm by giving chemotherapy first. In their series of 95 patients (Journal of Clinical Oncology 2019), those whose tumour responded to induction platinum-etoposide did better with definitive chemoradiotherapy than with surgery, while those who did not respond did better with surgery followed by radiotherapy or chemoradiotherapy, so the response to induction now decides the local treatment. Intensity-modulated or proton radiotherapy is used to spare the optic pathways and brain, and elective neck treatment is standard because nodal relapse is common. Metastatic disease is treated with platinum-etoposide as for neuroendocrine carcinoma, and PD-1 antibodies have produced responses in case series of SMARCB1-deficient and other sinonasal carcinomas. The IDH2 inhibitor enasidenib, approved for IDH2-mutant leukaemia, is being tested in IDH2-mutant sinonasal carcinoma, and EZH2 inhibitors, active in other SMARCB1-deficient tumours, are being explored for the SWI/SNF-deficient group. Five-year survival remains low overall, and referral to a skull base centre with molecular pathology is recommended for every patient.
A rare and aggressive sinonasal cancer of middle-aged adults, usually presenting as a large mass invading the orbit or skull base; historically most patients died within a few years, and induction chemotherapy has improved that.
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, Nasal cavity and paranasal sinus cancers (including esthesioneuroblastoma), 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)
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Biopsy with immunohistochemistry (cytokeratins, NUT, INI1, SMARCA4, neuroendocrine markers, EBER) and IDH2 testing; MRI and CT of the sinuses, skull base and neck; PET-CT.
Induction chemotherapy with cisplatin and etoposide (or docetaxel-platinum) for two to three cycles.
Definitive chemoradiotherapy with concurrent cisplatin (intensity-modulated or proton), including elective neck irradiation.
Surgical resection where feasible followed by radiotherapy or chemoradiotherapy.
Platinum-etoposide; PD-1 antibodies (pembrolizumab, nivolumab) on case series evidence; enasidenib for IDH2-mutant tumours in trials; EZH2 inhibitors for SWI/SNF-deficient carcinoma in trials.
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Sinonasal undifferentiated carcinoma is treated with induction platinum-etoposide, and the response decides whether the patient is spared radical surgery.
Sinonasal undifferentiated carcinoma can be confirmed by IDH2 testing or mutant-IDH immunohistochemistry rather than diagnosed by exclusion, and IDH2 inhibitors such as enasidenib became rational candidates for trials.
Query for this cancer: (TITLE:"Sinonasal undifferentiated carcinoma SNUC and SWI/SNF-deficient sinonasal carcinoma" OR ABSTRACT:"Sinonasal undifferentiated carcinoma SNUC and SWI/SNF-deficient sinonasal carcinoma" OR TITLE:"SNUC" OR ABSTRACT:"SNUC" OR TITLE:"IDH2-mutant sinonasal carcinoma" OR ABSTRACT:"IDH2-mutant sinonasal carcinoma" OR TITLE:"SMARCB1-deficient sinonasal carcinoma" OR ABSTRACT:"SMARCB1-deficient sinonasal carcinoma" OR TITLE:"SMARCA4-deficient sinonasal carcinoma" OR ABSTRACT:"SMARCA4-deficient sinonasal carcinoma" OR TITLE:"Undifferentiated carcinoma of the paranasal sinuses" OR ABSTRACT:"Undifferentiated carcinoma of the paranasal sinuses") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Sinonasal undifferentiated carcinoma (SNUC) and SWI/SNF-deficient sinonasal carcinoma, not a curated reading list.
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.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
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
Newly diagnosed? Read the first 60 days with Sinonasal undifferentiated carcinoma (SNUC) and SWI/SNF-deficient sinonasal carcinoma, then print the one-page appointment sheet with room for the answers.
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Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.