Juvenile nasopharyngeal angiofibroma

Case contributed by Dr Micheál Breen

Presentation

This 16 year old male patient presented with right sided nasal obstruction and rhinorrhoea.

Patient Data

Age: 16 years
Gender: Male

CT of the paranasal sinuses (contrast enhanced)

Modality: CT

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

Modality: 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 right external carotid artery

Modality: DSA (angiography)

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 behaviour.

It is almost exclusicely encountered in adolescent males1. 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 osseosus destruction is common. Orbital and intra-cranial extension can be seen at presentation which complicates treatment.

These lesions are highly vascular and biopsy is contraindicated due to the risk of haemmorhage. 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 choice2.

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Case Information

rID: 25926
Case created: 16th Nov 2013
Last edited: 3rd Dec 2015
Inclusion in quiz mode: Included

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