Clinical History
Clinical and imaging findings, together with gender and age of the patient, were suggestive for nasopharyngeal juvenile angiofibroma.
Imaging Findings
The previously healthy boy presented with complaints of progressive nasal obstruction, purulent rhinorrhea and headache.
Clinical examination revealed a lobulated soft tissue mass, filling up the posterior part of the right nasal cavity and entire nasopharynx. Conventional radiography of the paranasal sinuses (not shown) demonstrated opacification of the right nasal cavity and asymmetry of the maxillary sinuses.
CT scan of the maxillary sinuses, bone window setting shows a soft tissue mass occupying the nasopharyngeal space, the posterior right nasal cavity, right sphenoid sinus and right pterygopalatine fossa. Destruction and erosion of the surrounding bony structures and invasion of the medial wall of the right maxillary sinus is seen.
Gd-enhanced coronal SE T1-weighted MR image demonstrates inhomogeneous enhancement of the tumor, extending into the right sphenoid sinus and right sphenopalatine foramen.
On axial 3D-time of flight MR angiography (not shown), a flow signal is visible within the tumor. Hypertrophic right internal maxillary artery, which is presumably the major feeding artery is noted.
Selective arteriography of the right internal maxillary artery, lateral view, capillary phase (orientation is less obvious as the bony structures have been completely subtracted out) displays the reticular configuration of the small tumor vessels and intense, homogeneous blush.
Clinical and imaging findings, together with gender and age of the patient, were suggestive for nasopharyngeal juvenile angiofibroma. Histologic examination of the resected specimen confirmed the diagnosis.
Discussion
Nasopharyngeal angiofibroma, also called juvenile angiofibroma, is a benign, non-encapsulated, fibrovascular tumor, that grows very slowly. It is a common tumor of the nasopharynx, but represents only 0.05% of all head and neck neoplasms. The tumor has its onset in childhood with highest incidence in teenage males between 14 and 17 years old. Its etiology is unknown, but relation to sex hormones is strongly suggested.
From the nasopharynx, the tumor grows slowly into the pharynx, nasal fossa and nares. It may also originates in the pterygopalatine fossa. Cranially, the tumor may extend into the sphenoid sinus or even through the sinus into the sella turcica and cavernous sinus, involving the middle cranial fossa by extradural spread. Intracranial extension is less common. Finally, tumoral extension is also observed into the subtemporal space through the sphenomaxillary fissure, or through the inferior orbital fissure into the cranial floor.
CT scan is highly specific in identifying tumoral spread and skeletal changes. Transverse and coronal scanning should be performed in all cases. MRI reveals a mass of usually intermediate signal intensity on T1-and T2-weighted images, containing multiple signal voids representing the hypertrophic tumor vessels. MRI is also useful in the differentiation between tumoral extent into the sinuses and sinusitis. Preoperative angiography is mandatory for identification of the feeding vessels in view of preoperative embolization.
Differential Diagnosis List
Nasopharyngeal juvenile angiofibroma
Final Diagnosis
Nasopharyngeal juvenile angiofibroma
