Juvenile nasopharyngeal angiofibroma - extensive

Case contributed by Dr Mustafa Takesh

Presentation

History of recurrent epistaxis lasting 5-10 minutes, initially started on the left side and then turned bilateral.

Patient Data

Age: 16 years
Gender: Male

Selected images demonstrating a large mass lesion occupying the nasopharynx, sphenoid sinus as well as the posterior ethmoid air cells with lateral extension via left sphenopalatine fossa into the deep aspect of the left masticator space, anteriorly in the nasal chamber (in particular on the left side) as well as through the left superior and inferior orbital fissure into the orbital apex. There is deformity and decrease in size of the left maxillary sinus, secondary to the anterior displacement of the posterior wall.

Overall, findings are not compatible with an antrochoanal polyp, but suggestive of a large juvenile angiofibroma.

MRI images demonstrating extensive, strongly enhancing nasopharyngeal mass lesion with numerous flow voids. There is extension into the left infratemporal fossa with remodeling of the posterior wall of the maxillary sinus, as well as intraorbitally through the left inferior orbital fissure and orbital apex, with compression the left optic nerve. There are erosive changes in the skull base with intracranial/extradural extension in both the anterior and middle cranial fossa.  In addition, there is elevation of the pituitary gland and compression of left cavernous sinus, associated with encasement of the ICA cavernous segment, the later shows however preserved flow void.

On the TOF angiography, left maxillary artery appears to be the main feeder of this mass in the infratemporal fossa with marked enlargement of its anterior branch.

Overall, findings consistent with juvenile nasopharyngeal angiofibroma.

DSA (angiography)

Tumor embolization

Selected DSA images demonstrating the dominant supply to the tumor from the left internal maxillary artery.

EMBOLIZATION PROCEDURE: Injection of  Contour SE Microspheres size 500-700 mixed with saline and contrast media in the left internal maxillary artery with occlusion using two Axium prime 6X15 coils. 

POSTEMBOLIZATION FINDING:  Marked decrease in tumoral vascular supply with minimal persistent tumoral blush, no evidence of arteriovenous shunting. 

MRI

Postsurgical MRI

Selected images demonstrating post-surgical changes related to recent endoscopic resection of JNA with a large amount of packing material filling the surgical cavity. There is small residual tumor at the left inferior orbital fissure and cranial aspect of left infratemporal fossa.

Case Discussion

A 16-year-old male with a history of recurrent epistaxis, initially started on the left side and then becoming bilateral, progressed to being every three days with clots and congestion. In the past few weeks, he had headaches and weight loss.

The CT shows a large mass lesion occupying the nasopharynx, sphenoid sinus as well as the posterior ethmoid air cells with multilateral expansion, which indicates a large juvenile angiofibroma. The subsequent MRI demonstrated further typical features, including avid enhancement and flow voids.

The patient underwent embolization of the tumor and endoscopic resection 1 day later, the histology confirmed the diagnosis.

Juvenile nasopharyngeal angiofibroma is an uncommon locally destructive benign tumor with high risk of recurrence. There is a study showing that endoscopic approach is associated with a lower recurrence rate compared with open surgery.

This case shows the classic history and treatment approaches of JNA and points out the importance of interdisciplinary collaboration for the best management.

Case contributed by Dr Lucia Carpineta, Assistant Professor in Pediatric Radiology at McGill University.

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Case information

rID: 77167
Published: 7th May 2020
Last edited: 9th May 2020
Inclusion in quiz mode: Included
Institution: McGill University