They account for only 0. Clinical presentation The presentation is typical with obstructive symptoms, epistaxis , and chronic otomastoiditis due to obstruction of the Eustachian tube. Patients may present with life-threatening epistaxis. On examination, it may be seen as a pale reddish-blue mass. It is, as the name suggests, very vascular and a biopsy can sometimes be fatal. Pathology Juvenile nasopharyngeal angiofibromas are benign but highly vascular tumors.
|Published (Last):||17 October 2008|
|PDF File Size:||13.58 Mb|
|ePub File Size:||14.55 Mb|
|Price:||Free* [*Free Regsitration Required]|
E-mail: moc. This article has been cited by other articles in PMC. Abstract 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 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. It accounts for 0. Adolescents and young adults between 14 and 25 years are affected, and there is a distinct male predominance. In , 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 III is a calabash-like massive tumor lobe in the middle cranial fossa.
Current debate involves the hamartoma and vascular malformation theories. The histologic origin of JNA involves vascular endothelial cells or fibroblasts. Recent immunohistochemical and genetic studies throw some light on this topic. 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. 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. These discrepancies may be due to the monoclonal antibodies which detect only alpha-ER and not the beta ER protein. The hormonal influence in JNA remains controversial. He also complained of difficulty in breathing since 6—7 months. He had no noteworthy family history or past medical history.
Extraoral examination revealed normal appearing overlying skin. Intraoral swelling was diffuse, obliterating the vestibule and extending from the lateral incisor anteriorly till the pharynx posteriorly [ Figure 1 ].
Overlying mucosa appeared normal. On palpation, the swelling was firm and nontender.
Juvenile nasopharyngeal angiofibroma