Sinonasal tumors
Uncommon benign and malignant tumors of the nose and sinuses. One-sided blockage, bleeding or facial swelling in an adult needs an ENT check.
What it is
Benign and malignant tumors arising in the nasal cavity and paranasal sinuses, most often the maxillary sinus and ethmoid cells. They grow silently in air spaces, so early symptoms mimic sinusitis and many malignancies are diagnosed late, after invading the orbit, skull base or palate.
Who gets it
Sinonasal cancer represents under 1% of all cancers and a small fraction of head and neck cancers, mostly in men over 50. Squamous cell carcinoma is the most common malignancy; inverted papilloma is the most common benign epithelial tumor that matters clinically. Extranodal NK/T-cell lymphoma, nasal type, is EBV-related and comparatively more frequent in Asia and Latin America, including Colombia.
Causes
- Inverted papilloma: benign but locally aggressive tumor of the lateral nasal wall; HPV is detected in a subset; around 5 to 10% are associated with squamous cell carcinoma
- Squamous cell carcinoma: most often maxillary sinus or nasal cavity; tobacco, nickel and chromium exposure, malignant change of inverted papilloma
- Intestinal-type adenocarcinoma: ethmoid sinus, strongly linked to hardwood dust (furniture and carpentry work) and leather dust
- Olfactory neuroblastoma (esthesioneuroblastoma): neuroectodermal tumor of the olfactory epithelium at the cribriform plate; bimodal age peaks
- Mucosal melanoma: aggressive, nasal septum and lateral wall, older adults
- Extranodal NK/T-cell lymphoma, nasal type: Epstein-Barr virus associated, destructive midline lesion
- Others: adenoid cystic carcinoma (perineural spread), sinonasal undifferentiated carcinoma, sarcomas, metastases
Risk factors
- Occupational wood (especially hardwood) and leather dust
- Nickel, chromium, formaldehyde and textile dust exposure
- Tobacco smoking
- Inverted papilloma (risk of associated carcinoma and recurrence)
- Epstein-Barr virus infection (NK/T-cell lymphoma)
- Male sex and age over 50
Pathophysiology
Tumors expand within air-filled cavities until they obstruct drainage or erode bone. From the maxillary sinus they spread to the orbit (floor), cheek, palate and teeth, infratemporal fossa and pterygopalatine fossa; from the ethmoid and olfactory cleft they reach the orbit and the anterior cranial fossa through the cribriform plate. Ohngren's line (medial canthus to mandibular angle) separates anteroinferior maxillary tumors, with a better outlook, from posterosuperior ones that reach the skull base. Neck node spread is less common than in other head and neck cancers.
Classification
- Benign: inverted papilloma (Krouse staging), exophytic and oncocytic papillomas, osteoma, juvenile angiofibroma (nasopharynx, adolescent boys)
- Epithelial malignant: squamous cell carcinoma, intestinal-type adenocarcinoma, adenoid cystic carcinoma, sinonasal undifferentiated carcinoma
- Neuroectodermal: olfactory neuroblastoma (Kadish staging A to D, Hyams histologic grade), mucosal melanoma
- Hematolymphoid: extranodal NK/T-cell lymphoma, nasal type; diffuse large B-cell lymphoma
- TNM staging (AJCC) for maxillary sinus and nasal cavity–ethmoid carcinomas
Symptoms and signs
- Unilateral nasal obstruction that does not respond to treatment
- Unilateral bloody discharge or recurrent epistaxis
- Loss of smell (olfactory neuroblastoma)
- Facial swelling, pain or cheek numbness (infraorbital nerve)
- Loose teeth, ill-fitting denture or palatal mass
- Epiphora, proptosis, diplopia or visual loss with orbital spread
- Trismus (pterygoid invasion) and headache (skull base)
- NK/T-cell lymphoma: progressive necrotic ulceration of the nose and palate, septal or palatal perforation, fever, night sweats
Diagnosis
- Clinical
- Nasal endoscopy shows the mass (inverted papilloma: lobulated, firm, mulberry-like, from the lateral wall; melanoma: pigmented or not; neuroblastoma: high in the olfactory cleft). Examine eyes, cranial nerves, palate, teeth and neck nodes. A unilateral polyp in an adult must be sent for histology.
- Tests
- Biopsy with histology and immunohistochemistry is the diagnosis (for example S100, HMB-45 and Melan-A for melanoma; CD56, cytotoxic markers and EBV-encoded RNA for NK/T-cell lymphoma; neuroendocrine markers for neuroblastoma). Avoid biopsy of a possible vascular lesion before imaging.
- Imaging
- CT defines bone erosion (focal hyperostosis often marks the attachment of an inverted papilloma); MRI separates tumor from retained secretions and shows orbital, dural and perineural spread. PET-CT or neck and chest imaging to stage malignancies.
Differential diagnosis
- Chronic rhinosinusitis with nasal polyps (bilateral)
- Antrochoanal polyp
- Juvenile nasopharyngeal angiofibroma (adolescent boy with epistaxis)
- Invasive fungal sinusitis
- Granulomatosis with polyangiitis and cocaine-induced midline destruction
- Nasopharyngeal carcinoma
- Encephalocele or meningocele (never biopsy without imaging)
Goal of treatment
Complete removal with clear margins, preserving the eye, brain and function when possible, under a multidisciplinary head and neck team.
Treatment
- Surgical
- Inverted papilloma: complete endoscopic resection including the attachment site and the underlying bone; open approaches only when endoscopy cannot reach it.
- Surgical
- Sinonasal carcinomas, olfactory neuroblastoma and mucosal melanoma: surgical resection with clear margins (endoscopic, open maxillectomy or craniofacial resection depending on extent), sometimes with orbital exenteration.
- Procedure
- Postoperative radiotherapy for most malignancies, especially with close margins or advanced stage; radiotherapy with or without chemotherapy when the tumor is unresectable.
- Medical
- Extranodal NK/T-cell lymphoma: not treated with surgery; radiotherapy combined with asparaginase-based chemotherapy, led by hematology-oncology.
- Medical
- Mucosal melanoma with metastatic disease: immunotherapy with checkpoint inhibitors may be used by oncology.
- Follow-up
- Primary care role: refer any persistent unilateral nasal symptom or unilateral polyp to otolaryngology; long-term endoscopic follow-up after treatment because inverted papilloma and malignancies recur.
Complications
- Orbital invasion with vision loss
- Intracranial extension and CSF leak
- Recurrence (inverted papilloma, melanoma)
- Treatment effects: facial deformity, dry eye, epiphora, radiation injury to the optic pathway
- Distant metastasis (melanoma, adenoid cystic carcinoma)
Prognosis
Inverted papilloma is curable when completely removed but recurs if left behind. Overall five-year survival for sinonasal carcinoma is about half, worse in advanced stages; olfactory neuroblastoma has relatively good outcomes, while mucosal melanoma and NK/T-cell lymphoma carry a poor prognosis.
Test yourself
Think of the answer, then tap the question to see it.
A carpenter exposed to hardwood dust for decades develops unilateral nasal obstruction and epistaxis. Which tumor is most likely and where?
Intestinal-type adenocarcinoma of the ethmoid sinus.
What is the most common malignant tumor of the nasal cavity and paranasal sinuses?
Squamous cell carcinoma, most often in the maxillary sinus.
A benign sinonasal tumor of the lateral nasal wall that grows into the stroma, recurs and can harbor carcinoma is called?
Inverted (Schneiderian) papilloma.
A tumor arising from the cribriform plate in a patient with anosmia and unilateral obstruction, staged with the Kadish system, is?
Olfactory neuroblastoma (esthesioneuroblastoma).
A progressive destructive ulcer of the nose and palate with EBV-positive atypical cells is?
Extranodal NK/T-cell lymphoma, nasal type.
In ENT
- Other nasal problems: rhinolith, nasal myiasis, synechiae, antrochoanal polyp and CSF leak
- Endoscopic sinus surgery
In clinical skills
Connections
Educational information, not medical advice. For any symptom, see a health professional.
Sources: Esthesioneuroblastoma (StatPearls (NCBI Bookshelf), 2026); Extranodal NK-Cell Lymphoma (StatPearls (NCBI Bookshelf), 2023).
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Representative model, for educational purposes only.