Nasopharyngeal Tumours — Juvenile Nasopharyngeal Angiofibroma (JNA) and Nasopharyngeal Carcinoma

Both arise in the nasopharynx — the space behind the nasal cavity, above the soft palate. Its lateral wall carries the Eustachian tube openings, the torus tubarius and, behind them, the fossa of Rosenmüller (lateral pharyngeal recess). Tumours here hide from view, block the nose and the Eustachian tube, and spread into the skull base, so both present late with nasal and ear symptoms.

JNA is a histologically benign, highly vascular tumour that behaves aggressively by local invasion — into the turbinates, septum, medial pterygoid plate, pterygopalatine fossa and, in advanced cases, the infratemporal fossa, orbit, sphenoid and cavernous sinus. It makes up only about 0.05–0.5% of head and neck masses and occurs almost exclusively in adolescent males.

Its exact origin is debated: most sources place it at the sphenopalatine foramen and posterior nasal cavity; one theory links it to remnants of the first branchial arch. The main blood supply is the internal maxillary artery (a branch of the external carotid), although internal carotid feeders can also contribute. Hormonal influence is supported by androgen receptors in the tumour and reports of recurrence with exogenous testosterone.

Because early symptoms look like allergy or a simple nosebleed, diagnosis is often delayed. StatPearls advises that recurrent unprovoked epistaxis or persistent nasal obstruction that does not respond to allergy treatment in an adolescent boy should be evaluated promptly with endoscopy and imaging.

Diagnosis is clinical plus imaging. Contrast-enhanced CT shows an avidly enhancing mass in the posterior nasal cavity near the sphenopalatine foramen, often extending into the nasopharynx, pterygopalatine fossa and sinuses; CT is best for bone. MRI gives better soft-tissue contrast and shows spread into the cavernous or sphenoid sinus, orbit and skull base. Angiography maps the feeding vessels and allows embolisation.

The main differential in a young patient with nasal obstruction is an antrochoanal polyp, which arises in the maxillary sinus, passes through the ostium into the nose, rarely causes epistaxis and does not reach the sphenopalatine foramen or pterygopalatine fossa.

1. Pre-operative embolisation of the feeding vessels (gelatin sponge, particles or coils) to cut blood loss and surgical morbidity; it also delineates external- and internal-carotid feeders. Risks include facial palsy, infarction and cranial nerve injury.
2. Surgical excision — the treatment of choice. Endoscopic and combined endoscopic approaches are now widely used and cause less facial deformity than purely open approaches; open routes are kept for selected extensive tumours.
3. Radiotherapy for residual, recurrent or unresectable disease (up to a third of advanced Radkowski stage III tumours cannot be fully removed).
4. Hormonal therapy has a limited role — it risks feminisation in adolescent boys.

For JNA, surgeons may stage large resections — 'one bleed at a time' — dealing with each vascular territory separately; embolisation particles of about 300–500 µm are preferred to reduce the risk of non-target embolisation through external–internal carotid connections.

Nasopharyngeal carcinoma (NPC) is distinct from other head and neck squamous cancers. It has a striking geographic pattern: high incidence in Asia, especially southern China, and fewer than 1 case per 100,000 in the Americas and Europe, where it shows a bimodal age distribution. Males are affected more often than females.

• Epstein–Barr virus — most NPC is EBV-related; viral oncogenes LMP-1, LMP-2 and EBNA1 drive transformation.
• Genetic susceptibility — HLA variants that are more common in people of Chinese and South-East Asian ancestry; family history.
• Diet — preserved foods rich in nitrosamines, especially salted fish.
• Tobacco and alcohol — important in non-endemic areas.

The commonest presentation is a neck mass, usually unilateral, followed by headache and epistaxis. Other symptoms — nasal obstruction, reduced hearing, tinnitus, diplopia, facial numbness, trismus, ptosis or hoarseness — reflect where the tumour has spread. In any adult, a neck mass persisting more than 2 weeks should be presumed malignant until proven otherwise.

• Nodes — level II (jugulodigastric) nodes were traditionally thought commonest; modern imaging shows the retropharyngeal nodes are the most common metastatic site.
• Ear — tumour at the Eustachian tube causes unilateral middle-ear effusion; a one-sided effusion in an adult must prompt nasopharyngeal endoscopy.
• Cranial nerves — skull-base spread produces cranial neuropathies; a full cranial nerve examination is mandatory.

Evaluation: nasal endoscopy and endoscopic biopsy; CT, with MRI for soft-tissue extent and PET-CT for very small tumours that CT cannot resolve. Plasma EBV DNA is used for screening and surveillance in some endemic regions; high pre-treatment levels and detectable levels after treatment predict a worse outcome. Unilateral serous otitis media in an adult is the ENT link to chronic ear disease questions.

Radiotherapy is the essential treatment. Because the nasopharynx is so hard to reach, surgery is rarely used for the primary tumour. Intensity-modulated radiotherapy (IMRT), introduced in the 1990s, became the standard of care; it allows sparing of nearby structures such as the submandibular glands to reduce dry mouth.

Prognosis depends mainly on stage: survival is best in stage I and poorest in stage IV. Detectable EBV DNA after treatment is an established adverse prognostic marker, and high circulating EBV DNA is linked to poorer response, distant metastasis and death. Treatment and tumour effects to remember include hearing loss (Eustachian tube and middle-ear involvement) and dry mouth from salivary irradiation.

• Teenage boy with recurrent epistaxis and nasal block → JNA; investigation of choice → contrast CT/MRI; contraindicated → biopsy.
• Holman–Miller sign → anterior bowing of the posterior maxillary wall (JNA).
• Main feeder of JNA → internal maxillary artery.
• Most common site of NPC → fossa of Rosenmüller; most common histology → undifferentiated (WHO type 3).
• Virus → EBV; dietary risk → salted fish.
• Adult with unilateral serous otitis media → rule out NPC by nasopharyngoscopy.
• Trotter's triad → conductive deafness, V3 neuralgia, palatal palsy.
• Treatment of choice for NPC → radiotherapy (± cisplatin chemoradiation).