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Juvenile nasopharyngeal angiofibroma

Case contributed by Micheál Anthony Breen
Diagnosis certain

Presentation

Right-sided nasal obstruction and rhinorrhea.

Patient Data

Age: 16 years
Gender: Male

CT of the paranasal sinuses...

ct

CT of the paranasal sinuses contrast enhanced

There is a large mass extending from the posterior right nasopharynx extending posteriorly with osseous destruction to occupy a majority of the sphenoid sinus. The lesion enters and expands the right foramen rotundum. The superolateral margin of the right sphenoid sinus shows a subtle bony defect adjacent to the mass but there is no evidence of intracranial extension. The mass extends into, fills and expands the right pterygopalatine fossa. Laterally, it extends through the pterygomaxillary fissure into the masticator space. There is bowing of the medial wall of the right orbit and the mass erodes portions of the posterior and medial wall of the right maxillary sinus. 

MRI of the paranasal sinuses

mri

Again noted is a large mass extending from the right posterior nasopharynx into the sphenoid sinus. It expands the pterygopalatine fossa on the right filling the right retroantral fat pad, extends laterally through the pterygomaxillary fissure into the subjacent masticator space. The mass is isointense on T1, heterogeneously hyperintense on STIR, containing some internal flow voids, and demonstrates marked contrast enhancement on the postcontrast imaging. There is opacification of the right maxillary sinus with bloody or proteinaceous fluid. Remainder of the paranasal sinuses and mastoid air cells are clear. 

DSA of the Rt external...

dsa

DSA of the Rt external carotid artery

Selective injection of the right external carotid artery demonstrates a hypervascular tumor blush supplied by posterior sphenopalatine branches of the right right internal maxillary artery.  

Control angiography after embolization of the distal right internal maxillary artery demonstrates minimal opacification of the previously seen hypervascular blush.

Case Discussion

This is a classical presentation of juvenile nasopharyngeal angiofibroma. This is a histologically benign lesion with very aggressive local behavior.

It is almost exclusively encountered in adolescent males 1. The most common presenting symptom is painless nasal obstruction or epistaxis; however, other symptoms may develop depending on the size and extent of the tumor mass. 

The lesion tends to spread via the pterygopalatine fossa and osseous destruction is common. Orbital and intracranial extension can be seen at presentation which complicates treatment.

These lesions are highly vascular and biopsy is contraindicated due to the risk of hemorrhage. CT or MRI have important roles in assessing the tumor mass and extension and assist with treatment planning. DSA elegantly demonstrates the vascular supply and allows pre-operative embolization of the feeder vessels. Post-embolization surgical resection is the treatment of choice 2.

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