nasopharyngeal angiofibromas are relatively rare tumors that represent 0.5% of all head and neck neoplasms and they predominantly occur in adolescent males ( 1 ) .
this type of tumor originates from the region of the sphenopalatine foramen . in most cases
, imaging studies will demonstrate a widening of the pterygopalatine fossa and anterior bowing of the posterior wall of the maxillary sinus ( 2 ) .
i describe an extra - nasopharyngeal angiofibroma arising from the parapharyngeal space in a middle - aged woman without involvement of the sphenopalatine foramen or the nasopharynx .
a 53-year - old woman presented with a diffuse anterior neck swelling of one - month duration . the patient had not undergone any previous surgery .
ultrasonography ( us ) showed diffuse goiter with multiple thyroid nodules . a homogeneous and hypoechoic solid mass , adjacent to the upper margin of the right submandibular gland with focal flow signals on color doppler sonography within the mass was detected ( figure 1 ) . a computed tomography ( ct ) scan revealed a circumscribed mass , hypodense to the masseter muscle and hyperdense to the parotid gland in the right parapharyngeal space ( figure 2 a - c ) .
after intravenous contrast administration , the mass , 1.7  1.7  1.6 cm in size , demonstrated homogeneous enhancement without infiltration to the adjacent muscle . at that time
enlargement of the bilateral thyroid lobes with multiple non - enhancing hypodense and calcified thyroid nodules was also noted . under general anesthesia
after surgery , the patient revealed an uneventful postoperative course . at the three - year follow - up examination ,
angiofibromas are histologically benign , but potentially locally destructive fibro - vascular tumors that account for less than 0.5% of all head and neck neoplasms ( 3 ) .
they originate from the region of the sphenopalatine foramen and commonly spread to the sphenoid sinus , the nasal cavity , and the pterygopalatine foramen ( 1 ) .
angiofibromas that do not originate from the area around the sphenopalatine region are very rare ( 1 ) .
the primary site of extra - nasopharyngeal angiofibromas varies and can include the ethmoid and sphenoid sinuses , the nasal septum , the tonsils , the ears , the trachea , the larynx , and the infratemporal fossa ( 1 ) .
the most common site of extra - nasopharyngeal angiofiroma is the maxillary sinus . in the recent literature ,
there were five more cases of extra - nasopharyngeal angiofibromas reported in the literature since then ( 5 - 9 ) .
only one parapharyngeal angiofibroma was reported in a 25-year - old male in the english literature ( 10 ) .
they reported homogeneous enhancement on ct and hypervascularity on conventional angiogram in the left parapharyngeal mass .
i report the very rare case of an extra - nasopharyngeal angiofibroma arising from the right parapharyngeal space in a middle - aged woman . to the best of our knowledge
, this represents the second demonstrated case of an angiofibroma originating in such a location .
extra - nasopharyngeal angiofibromas occur more commonly in females and tend to develop at an older age ( 11 ) .
the male - to - female ratio is roughly 3:1 and the mean age at presentation is 22 years .
clinical manifestations of extra - nasopharyngeal angiofibromas are variable including headache , hoarseness , dysphagia , dyspnea , stridor , and pain according to their location ( 4 , 11 ) .
extra - nasopharyngeal angiofibromas may have less specific and confusing clinical symptoms and diagnosis may be delayed ( 4 ) .
enlargement of the sphenopalatine foramen with an intensely enhancing mass is the typical pathognomonic radiological finding , especially in young boys ( 1 ) . in the patient with characteristic radiological findings and typical clinical symptoms of nasopharyngeal angiofibromas , preoperative biopsy
extra - nasopharyngeal angiofibromas are harder to diagnose radiologically due to their various locations ( 4 ) .
unlike nasopharyngeal angiofibromas , lack of hypervascularity on conventional angiograms does not exclude the diagnosis of extra - nasopharyngeal angiofibromas ( 2 ) .
an axial ct showed well enhancing mass in the right parapharyngeal space without perilesional infiltration .
the mass was thought to be a minor salivary gland origin tumor or a paraganglioma , hemangiopericytoma , solitary fibrous tumor or neurogenic tumor , preoperatively .
differential diagnoses of parapharyngeal masses may also include parapharyngeal space metastatic lymph node , lymphoma , inflammatory pseudotumor , tuberculous and fungal infections ( 12 ) .
the most acceptable treatment for angiofibroma is endovascular embolization and then surgical excision ( 13 ) .
nasopharyngeal angiofibroma seems to have a poorer prognosis than an extra - nasopharyngeal lesion due to inadequate surgical exposure ; consequently , it may result in a higher recurrence rate ( 3 ) . in the present case ,
complete surgical resection was performed without preoperative embolization . on follow - up after a period of 3 years , there was no tumor recurrence .
 nasopharyngeal angiofibroma is a relatively uncommon vascular tumor affecting adolescent males and it characteristically originates in the posterior lateral wall of the nasopharynx . 
 primary extra - nasopharyngeal angiofibroma is very rare . 
 here , i present a case of angiofibroma of the parapharyngeal space in a 53-year - old woman with ct and sonographic findings . 
1. Introduction
2. Case Presentation
3. Discussion