it accounts for 0.5% of all head and neck tumors occurring in 1 of 150,000 individuals . adolescents and young adults between 14 and 25 years are affected , and there is a distinct male predominance . recent reports suggest that the tumor is more common in the indian subcontinent than in the west . hippocrates first described this tumor in the 5 century b.c . in 1940 , friedberg called it juvenile angiofibroma . however , since a vast majority of the cases do occur between 14 and 25 years of age , this term is retained . the prevalence in males may be explained by high androgen receptor ( ar ) expression suggesting that jna is androgen dependent . type i includes lesions fundamentally localized to the nasal cavity , paranasal sinus , nasopharynx , or pterygopalatine fossa . type ii is a jna extending into the infratemporal fossa , buccal region , or orbital cavity with anterior and/or minimal middle cranial fossa extension but intact dura mater . type iii is a calabash - like massive tumor lobe in the middle cranial fossa . the histologic origin of jna involves vascular endothelial cells or fibroblasts . whether both components proliferate and grow together or one component is responsible for the growth and other efforts to determine the pathogenesis of the tumor have been done by studying the expression of various growth factors and oncogenes such as c - kit and c - myc . significantly higher immune staining with cd34 , vascular endothelial growth factor , flt-1 and flk-1 in jnf , when compared to orbital cavernous hemangiomas , indicates its vasoproliferative nature . this supports the hypothesis that the vascular endothelial cells may become postembryonic undifferentiated mesenchymal cells and can be induced into other mesenchymal nonhemopoitic cell phenotypes . loss of expression of gstm 1 ( null genotype ) is seen in this tumor . jna 's sex selectivity and the relatively young age at diagnosis suggest that its development is hormone dependent . several authors studied ars , progesterone receptors , and estrogen receptors ( ers ) in jna with discrepancies in the results . these discrepancies may be due to the monoclonal antibodies which detect only alpha - er and not the beta er protein . in spite of the reports of hormonal disorders in patients with jna and the presence of ar and/or ers in tumor tissues , no apparent alterations of serum hormone levels a 17-year - old male patient presented with a painless , progressive swelling in the upper jaw since 2 weeks . intraoral swelling was diffuse , obliterating the vestibule and extending from the lateral incisor anteriorly till the pharynx posteriorly [ figure 1 ] . computed tomography ( ct ) scan showed the presence of a soft tissue mass involving the maxillary sinus , nasal cavity , and nasopharynx . the mass extended superiorly from the skull base till the maxillary tuberosity and anteroposteriorly from nasal cavity till posterior wall of maxillary sinus [ figures 2 and 3 ] . intraoral view showing swelling on the upper left side of the maxilla and obliteration of the vestibule sagittal section in computed tomography scans showing site and extent of the lesion axial section in computed tomography demonstrating obliteration of the nasal cavity and maxillary sinus provisional diagnosis of nasopharyngeal angiofibroma , soft tissue tumor , nasopharyngeal polyp or vascular tumor was made . the excised gross specimen was soft to firm in consistency , white to yellow with darker vascular areas . no encapsulation was noted [ figure 4 ] . gross specimen showing tumor mass with vascular areas histopathological examination revealed a proliferative connective tissue stroma interspersed with a thick vascular network . numerous blood vessels with irregular size and shape were seen with a single lining endothelial cell layer . blood vessels were seen in large number at the periphery of the lesion [ figure 5 ] . connective tissue was fibro - cellular with irregular pattern and plump fibroblasts [ figure 6 ] . photomicrograph showing fibrocellular stroma and numerous blood vessels at the periphery ( h&e stain , 40 ) photomicrograph showing a staghorn appearance of the blood vessels ( h&e stain , 100 ) distribution of mast cells in the lesion ( toluidine blue stain , 40 ) although benign , it is a locally aggressive tumor and invades the surrounding tissues and even bone through pressure resorption . this tumor originates in the lateral wall of the nasal cavity , close to the superior border of the sphenopalatine foramen . the growth initiates in the submucosa of the floor of the nasopharynx extend to the nasal septum and the posterior space of the nose and causes airway obstruction . continuous growth involves the sphenoidal sinus , nasal fossa and middle turbinate , pterygomaxillary fossa and the posterior wall of the maxillary sinus as seen in the present case . eventually , the tumor may invade the infratemporal fossa and the middle cranial fossa . isolated case reports of angiofibromas arising outside the nasopharynx are noted , most commonly in the maxillary sinus ( 32% ) and ethmoid sinus ( 10% ) . jna is classified as type i when the tumor is restricted to the nasal cavity and the nasopharynx without bone destruction , type ii when the tumor invades the pterygomaxillary fossa and maxillary , sphenoidal and ethmoid sinuses with bone destruction , type iii when the tumor invades the infratemporal fossa , the orbit , and the parasellar region but remains lateral to the cavernous sinus and type iv when the tumor invades the cavernous sinus , the optic chiasma and the pituitary fossa . as this tumor is aggressive and expansile , it invades adjacent structures causing further symptoms . impaired eustachian tube function , facial deformity , proptosis and changes in visual acuity may be seen . angiofibromas originating outside the nasopharynx may appear as an intraoral mass in the retromolar or buccal space area . diagnosis of jna is made by complete history , clinical examination , radiography , nasal endoscopy ; and specialized imaging techniques such as arteriography , ct , and magnetic resonance imaging ( mri ) . these techniques help to establish the exact site , extension and relation of the tumor to the adjacent structures such as blood vessels and nerves . avid enhancement is noted on contrast - enhanced ct . at the time of diagnosis , the mass classically involves the pterygopalatine fossa . in this location , it produces widening of the pterygopalatine fossa , inferior orbital and pterygomaxillary fissures and bowing of the posterior wall of the maxillary antrum . bony erosion of the nasal cavity , hard palate and pterygoid plates is also common . anterior bowing of the posterior maxillary wall , due to invasion of the pterygomaxillary space on axial ct , known as the holman - miller sign is one of the characteristic findings . coronal ct images show the relationship between the tumor and surrounding structures based on which the staging can be done . the lesion shows low signal intensity in t1-weighted images but heterogeneous intermediate signal intensity in t2-weighted images . these features along with the specific age and sex predilection can help in differentiating jna from other nasopharyngeal lesions . mri is used posttreatment to detect residual or recurrent tumor mass and to monitor the effects of radiotherapy . the location and size of the tumor and feeding vessels are clearly demonstrated by this technique . the vascular supply to jnas is primarily from distal internal maxillary artery branches , particularly the sphenopalatine , descending palatine and posterior superior alveolar branches . these lesions include inflammatory polyps , angiomatous polyps , nasopharyngeal cysts and carcinomas , soft tissue neoplasms such as papilloma , lymphoma , neurofibroma , maxillary malignancies , nasal fossa esthesioneuroblastoma , adenoid hypertrophy , cervical vertebrae cordomas and retropharyngeal ganglia tuberculosis . a careful history noting , age , sex and location of the lesion may help to differentiate jna from other lesions . the final diagnosis is achieved by histopathologic examination of tissue sections either on incisional or excisional biopsy . the color depends on the vascular component and may vary from pale white in less vascular lesions to a pink and wine colored mass in highly vascularized ones . histopathologically jna shows a fibrocellular stroma with spindle cells and haphazardly arranged collagen interspersed with an irregular vascular pattern . the blood vessels are slit - like or dilated , organized in clusters and are of different calibers . a higher density toward the periphery the muscular lining of the blood vessel is absent in small and incomplete in larger vessels . beham et al . in 1997 proved the spindle cells of jna to be fibroblasts and not myofibroblasts . however , myxoid areas were not present in our case , they can be seen interspersed within the tumor . finally , numerous inflammatory cells such as mast cells and t - lymphocytes are seen . advanced lesions with orbital and intracranial extension are difficult to treat and may recur often . when diagnosed early the patients are treated with a combination of preoperative embolization and surgical resection providing a good prognosis . recurrent tumors must be managed individually taking into account location , age , complications and the possibility of spontaneous resolution . several reports suggest that jna regresses over time . a wait and watch policy with periodic imaging recurrence rates depend on the clinical stage of the tumor but are generally higher in patients with anterior and/or posterior lateral extension than in patients with anterior lateral extension only . jna is an uncommon , highly vascular , locally invasive , unencapsulated tumor with a distinct predilection for an origin in the nasopharynx of adolescent males . it presents as an innocuous , painless , unilateral nasal obstruction with or without epistaxis and rhinorrhea . diagnosis is arrived at by clinical examination , radiography , nasal endoscopy and specialized imaging techniques such as ct scan and mri . the classification and clinical staging is relatively easy and helps to plan treatment and determine prognosis . early diagnosis , accurate staging and adequate treatment are essential in the management of this lesion .
juvenile nasopharyngeal angiofibroma ( jna ) is a rare benign tumor arising predominantly in the nasopharynx of adolescent males . 
 it is an aggressive neoplasm and shows a propensity for destructive local spread often extending to the base of the skull and into the cranium . 
 clinically , however , it is obscure with painless , progressive unilateral nasal obstruction being the common presenting symptom with or without epistaxis and rhinorrhea . 
 diagnosis of jna is made by complete history , clinical examination , radiography , nasal endoscopy and by using specialized imaging techniques such as arteriography , computer tomography and magnetic resonance imaging . 
 histopathology reveals a fibrocellular stroma with spindle cells and haphazard arrangement of collagen interspersed with an irregular vascular pattern . 
 a case report of jna with rare intra - oral manifestation in a 17-year - old male patient is presented in the article . 
 jna being an aggressive tumor may recur posttreatment . 
 thus , early diagnosis , accurate staging , and adequate treatment are essential in the management of this lesion .