Juvenile nasopharyngeal angiofibroma

Case contributed by Ryan Thibodeau
Diagnosis certain

Presentation

Progressive nasal fullness and epistaxis. CT at outside hospital revealed a sinonasal mass.

Patient Data

Age: 13 years
Gender: Male

There is an intensely enhancing mass centered in the left nasal cavity with expansion of the left sphenopalatine foramen. The lesion extends into the nasopharyngeal airway and causes mass effect on the nasopharyngeal adenoids. It extends posteriorly to abut the region of the anteroinferior aspect of the clivus but is separated from the clivus by the prevertebral fascia. The lesion abuts the inferomedial aspect of the left orbital wall, without extension into the orbit. There is no intracranial extension.

Multiple flow voids within the mass are seen. There is inspissated mucus in the sphenoid sinuses, left ethmoid, and left maxillary sinus.

There is significant tumor blush seen on both the frontal and lateral views when cannulating the external carotid artery.

Case Discussion

This is a juvenile nasopharyngeal angiofibroma.

This patient underwent endoscopic endonasal resection of their angiofibroma that involved the left nasal cavity and infratemporal fossa. Gross pathologic examination revealed rubbery, tan-pink fibrous tissue. Histopathologic examination of these tissues revealed an admixture of blood vessels of variable sizes within a fibrous stroma of variable cellularity, consistent with angiofibroma. The tumor stained positively for beta catenin and scattered positivity for factor XIIIa. The vessels within the tumor were highlighted by CD34, actin, and smooth muscle actin. S100 was negative and BCL2 had weak, patchy staining limited to the endothelial cells. 

The patient did excellent postoperatively with immediate improvement of nasal breathing. There was some residual nasal discharge in the month after surgery, but the patient denied pain, paresthesia, diplopia, and blurry vision. He has continued to follow with otolaryngology for routine surveillance and has showed no signs of recurrence.

Co-authors:
Mason Soeder

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