Juvenile nasopharyngeal angiofibromas (JNA) are a rare benign but locally aggressive vascular tumor. Epidemiology Juvenile nasopharyngeal angiofibromas. Introduction: Juvenile nasopharyngeal angiofibroma (NAJ) is a tumor with .. Acessos Cirúrgicos no Angiofibroma Nasofaríngeo Juvenil – Relato de caso e. Juvenile nasopharyngeal angiofibroma (JNA) is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the.
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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 ahgiofibroma to the base of the skull and into the cranium. Clinically, juevnil, 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 juvenl 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 jhvenil essential in the management of this lesion. Juvenile nasopharyngeal angiofibroma JNA is a benign neoplasm of the nasopharynx.
It accounts for 0. Adolescents and young adults between 14 and 25 years are affected, and there is a distinct male predominance. Hippocrates first described this tumor in the 5 th century B.
InFriedberg 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.
Based on the clinical and radiological features, JNA is classified into three types. Type I includes lesions fundamentally localized to the nasal cavity, paranasal sinus, nasopharynx, or pterygopalatine fossa.
Endoscopic Surgery of Nasopharyngeal Angiofibroma
Type III is a calabash-like massive tumor lobe in the middle cranial fossa. The origin and development of JNA is not fully understood. Current debate involves the hamartoma and vascular angiofibro,a theories. Juenil 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 juvnil 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. JNA’s sex selectivity and the relatively young age at diagnosis suggest that its development is hormone dependent. 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. A year-old male patient presented with a painless, progressive swelling in the upper jaw since 2 agiofibroma. 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. Intraoral view showing swelling on the upper left side of the maxilla and jjvenil of the vestibule.
Computed tomography CT scan showed the presence of a soft tissue mass involving the maxillary sinus, nasal cavity, and nasopharynx. 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. Surgical excision was done with moderate intraoperative bleeding.
The excised gross specimen was soft to firm in consistency, white to yellow with darker vascular areas. No encapsulation was noted [ Figure 4 ]. 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 aangiofibroma ].
Connective tissue was fibro-cellular with irregular pattern and plump fibroblasts [ Aangiofibroma 6 ]. Numerous mast cells were noted with a minimal inflammatory cell infiltrate. Figure 7 shows mast cells in toluidine blue stain. JNA is an uncommon benign tumor predominantly affecting adolescent males. Although benign, it is a locally aggressive tumor and invades angioibroma 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. 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.
They develop at a slightly older age and occur amgiofibroma commonly in women. JNA is benign but locally destructive. This may be attributed to a rich vasculature and lack of encapsulation.
It impinges on adjacent structures and causes pressure erosion juvebil bone. Fisch classification, however, is currently accepted. 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 agiofibroma 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 ajgiofibroma sinus, angioifbroma optic chiasma and the pituitary fossa.
JNA classically presents as a painless, progressive unilateral nasal anguofibroma. Epistaxis, rhinorrhea and pain may also be seen. Clinical examination reveals a firm and friable mass angiofibtoma the nasopharynx and nose. 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. Invasion of the intracranial region may lead to cranial nerve palsy.
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 juvenol, radiography, nasal endoscopy; and specialized imaging techniques such as arteriography, CT, and magnetic resonance imaging MRI.
These techniques angiofihroma to establish the exact site, extension and relation of the tumor to the adjacent structures such as blood vessels and nerves. This makes it possible to precisely stage JNA.
CT images show a heterodense mass that is centered in the angifoibroma foramen. 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.
Accordingly, the treatment options, operative approach and angipfibroma can be determined. MRI is superior to CT for detecting soft tissue extension of the tumor intracranially.
Contrast enhanced MRIs are used to achieve avid enhancement jhvenil flow voids. These features angipfibroma 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. Angiography is a useful adjunct in the diagnosis of vascular tumors. 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. The main clinical presentation of JNA is unilateral nasal obstruction with or without epistaxis. Any lesion with this presentation may be confused with JNA. 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 juveil cordomas and retropharyngeal ganglia tuberculosis.
The final diagnosis is achieved by histopathologic examination of tissue sections either on incisional or excisional biopsy. Macroscopically this tumor appears as a rounded, circumscribed, noncapsulated mucosa covered mass. The color depends on the vascular component and may vary from pale white juvenul 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.
Angiofibroma nasofaríngeo juvenil
The blood vessels are slit-like or dilated, organized in clusters and are of different calibers. A higher density toward the periphery is noted, which was seen in the present jvuenil too. The muscular lining of the blood vessel is absent in small and incomplete in larger vessels. A typical staghorn type appearance is seen. Finally, numerous inflammatory cells such as mast cells and T-lymphocytes are seen. Early diagnosis and treatment are required for a good prognosis in JNA. Unfortunately, juveni is difficult due to innocuous presenting symptoms.
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.