Presentation
Progressive diplopia, blurry vision, and headache.
Patient Data
There is heterogeneously enhancing soft tissue centered within the sphenoid sinus with remodeling of the sinus walls. There is minimal soft tissue protruding into the right orbital apex. Anteriorly, the mass extends into the ethmoid sinuses and nasal cavity. There is widening of the left sphenopalatine foramen and left pterygopalatine fossa. Within the left retromaxillary fissure, there is a soft tissue mass eroding the posterior wall of the left maxillary sinus. There is a soft tissue component within the left masticator and parapharyngeal space.
Superiorly, there is destruction of the anterior skull base, with an intracranial soft tissue component. There is superior displacement of the gyrus recti. There is soft tissue within the left cavernous sinus, which extends anteriorly and widens the left superior and inferior orbital fissures.
There is a large, intensely enhancing mass lesion with marked osseous remodeling and/or dehiscence, centered within and totally replacing the sphenoid sinus with multicompartment extension. There are several serpiginous flow-voids within the mass reflecting enlarged
feeding vessels. There is lateral extension of the mass lesion with expansion of the left
pterygopalatine fossa and left inferior orbital fissure. There is superior displacement of the left intraorbital optic nerve and left medial rectus muscle.
There is extension into the anterior cranial fossa with marked dehiscence of the planum sphenoidale and sellar floor. There is extension into the left middle cranial and left infratemporal fossa/masticator space.
There is sinonasal extension primarily into the right maxillary sinus with near total opacification. There is left middle ear/mastoid effusion.
There is a large tumor blush from the internal maxillary artery (primary) as well as the inferolateral trunk.
Case Discussion
This is a case of a juvenile nasopharyngeal angiofibroma.
This patient was initially treated with preoperative embolization before being taken to the operating room for extensive surgical interventions including bilateral maxillary antrostomy, bilateral total ethmoidectomy, bilateral sphenoidotomy, left frontal sinusotomy, posterior septectomy, and bilateral middle and superior turbinate resections. Gross pathologic examination of the specimens revealed rubbery, irregular, tan-pink tissues which varied in size. Histopathologic examination of these tissues revealed inflamed sinonasal mucosa and fibrocollagenous stromal proliferation, consistent with angiofibroma. The tumor stained positively on immunohistochemical stain for beta-catenin, with weak positivity for androgen receptor and CD117, and negative staining for CD34 and ER.
The patient initially did well postoperatively but was found to have residual tumor on a follow-up MRI. Further endoscopic resection including a left-sided medial maxillectomy was performed. Postoperative MRI revealed that the residual tumor had been removed. The patient noted immediate and significant improvement in vision, but still endorsed mild blurry vision of the left eye. The patient continues to follow with otolaryngology for oncologic surveillance.
Co-authors:
Mason Soeder
Daniel Gewolb, MD
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