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Sinonasal malignancy

What's new

The European Society of Medical Oncology (ESMO) and the European Reference Network on Rare Adult Solid Cancers (EURACAN) have published a new guideline for sinonasal malignancies, covering various histological subtypes including squamous cell carcinoma, adenocarcinoma, melanoma, esthesioneuroblastoma, and neuroendocrine tumors. Prompt complete ENT examination, including nasal endoscopy, is recommended for patients with sinonasal symptoms lasting ≥3 weeks. Surgery is recommended only when complete resection is considered feasible. Tumors are deemed unresectable when any of the following sites are involved: orbital apex, cavernous sinus or optic chiasm, encasement of the internal carotid artery, massive brain invasion with perilesional edema, or involvement of major vessels. Intensity-modulated radiotherapy is the standard choice for both definitive and postoperative radiotherapy. .

Background

Overview

Definition
Sinonasal malignancies are a heterogeneous group of rare malignant tumors arising from the nasal cavity or paranasal sinuses, encompassing various histological subtypes, including SCC, adenocarcinoma, melanoma, esthesioneuroblastoma, and NETs.
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Pathophysiology
Sinonasal malignancies arise from a variety of histologically and molecularly distinct carcinomas originating in the nasal cavity, paranasal sinuses, or skull base. SCC, accounting for 50-75% of cases, includes keratinizing and non-keratinizing variants, with the latter frequently associated with high-risk HPV, particularly types 16 and 18. Molecular alterations, such as DEK::AFF2 fusions in non-keratinizing SCC, IDH2 mutations in sinonasal undifferentiated carcinoma, and EGFR exon 20 mutations in inverted papilloma-related SCC, aid in defining subtypes and may influence prognosis. Other high-grade malignancies include SWI/SNF-deficient carcinomas (SMARCB1 or SMARCA4 loss), NUT carcinomas (NUTM1 rearrangements), and HPV-related multiphenotypic sinonasal carcinoma, which, despite aggressive histology, often has a more favorable clinical course. Genetic mutations in TP53, CDKN2A, CTNNB1, and PIK3CA are variably seen across subtypes, supporting the heterogeneous and molecularly complex nature of these tumors.
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Epidemiology
The incidence of sinonasal malignancies is estimated at < 10 cases per million annually (5-9 in males, 2-5 in females), accounting for < 5% of all head and neck cancers. The nasal cavity is the most frequently affected site, followed by the maxillary, ethmoid, sphenoid, and frontal sinuses.
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Risk factors
Risk factors include occupational exposures (such as wood, textile, leather, flour, nickel, and chromium dust), tobacco smoking, chronic sinonasal inflammation, previous radiation exposure, and HPV infection.
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Disease course
Patients usually present with nonspecific symptoms such as nasal obstruction, epistaxis, facial pain or swelling, anosmia, and persistent sinusitis-like symptoms, often leading to delayed diagnosis. Advanced lesions frequently invade adjacent structures, including the orbit, skull base, and cranial nerves, resulting in significant morbidity.
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Prognosis and risk of recurrence
Prognosis varies widely based on histological type, stage at diagnosis, and extent of local invasion, often necessitating aggressive multimodal therapy involving surgery, radiation, and chemotherapy.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of sinonasal malignancy are prepared by our editorial team based on guidelines from the European Reference Network on Rare Adult Solid Cancers (EURACAN/ESMO 2025), the American Society of Pain and Neuroscience (ASPN 2021), the European Society of Medical Oncology (ESMO 2020), the Multinational Association of Supportive Care in Cancer (MASCC/ISOO 2020), ...
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Diagnostic investigations

Clinical examination: as per ESMO/EURACAN 2025 guidelines, perform a complete ear, nose, and throat examination, including nasal endoscopy, in patients with sinonasal symptoms lasting ≥ 3 weeks.
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  • Imaging for staging

Diagnostic procedures

Histopathology: as per ESMO/EURACAN 2025 guidelines, confirm the diagnosis with radiological imaging and histopathology supplemented by immunohistochemistry and molecular studies.
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Medical management

General principles: as per ESMO/EURACAN 2025 guidelines, manage patients with sinonasal malignancy by a multidisciplinary team in high-volume centers.
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  • Management of local/locoregional disease (neoadjuvant chemotherapy)

  • Management of local/locoregional disease (surgery)

  • Management of local/locoregional disease (adjuvant therapy)

  • Management of local/locoregional disease (radiotherapy)

  • Management of metastatic disease

  • Management of recurrence

  • Prevention of oral mucositis (general principles)

  • Prevention of oral mucositis (oral cryotherapy)

  • Prevention of oral mucositis (intraoral photobiomodulation therapy)

  • Prevention of oral mucositis (mouthwashes)

  • Prevention of oral mucositis (oral glutamine)

  • Prevention of oral mucositis (recombinant keratinocyte growth factor 1)

  • Prevention of oral mucositis (chewing gum)

  • Prevention of oral mucositis (honey)

  • Prevention of oral mucositis (therapies with no evidence for benefit)

  • Management of oral mucositis (morphine)

  • Management of oral mucositis (ketamine)

  • Management of oral mucositis (pilocarpine)

  • Management of oral mucositis (sucralfate)

  • Management of oral mucositis (antibiotics)

  • Management of oral mucositis (hyperbaric oxygen therapy)

  • Management of oral mucositis (counseling)

Follow-up and surveillance

Clinical follow-up: as per ESMO/EURACAN 2025 guidelines, consider initiating follow-up planning within 3 months after the end of treatment.
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  • Imaging follow-up