Juvenile Angiofibroma
Juvenile nasopharyngeal angiofibroma is a histologically benign yet locally aggressive vascular head and neck tumor. Angiofibroma affects almost exclusively adolescent boys, but has been reported in women and elderly patients on rare occasions. It has a firm, nodular texture with a sessile base. It is nonencapsulated and may attach to invaded structures. Early tumors are localized to the posterior nares. With growth the lesion simultaneously expands anteriorly in the nasal cavity and posteriorly and superiorly in the nasopharynx. Posterior erosion can invade the sphenoid sinus and sella turcica. As it expands it tends to extend through the sphenopalatine foramen to involve the pterygopalatine fossa; there it exerts pressure on the surrounding bone. This behavior accounts for the radiologic finding of anterior bowing of the posterior wall of the maxillary sinus , as well as for the distortion and posterior displacement of the pterygoid plates. It is important to highlight the fact that maxillary sinus invasion, when it does occur, does so via this route and rarely if ever from the nasal cavity outward laterally. As the lesion continues to expand, it does so by escaping further laterally via the pterygomaxillary fissure into the temporal and infratemporal fossae. The latter will present as an obvious cheek mass of the face, or as intraoral buccal mass. By the time a mass appears above the zygoma, the lesion has invariably invaded the inferior orbital fissure from which it expands into the lower end of the superior orbital fissure, where these two join at the posteriosuperior end of the pterygopalatine fossa.
This is manifest clinically as proptosis. The lesion can extend intracranially by one of two routes.The most common is via a continuation of growth through the expanded orbital fissures; from which it comes to lie lateral to the cavernous sinus. Histologic serial sections of fetal heads have confirmed the presence of large endothelial lined spaces in the region of the sphenopalatine foramen and base of the pterygoid plates in both males and females. Because of the development of this tumor in males near puberty a hormonal response by the tumor is presumed. Receptor studies in the past have revealed no estrogen or progesterone receptors in tumor specimens. Bretani, et al.Juvenile nasopharyngeal angiofibromaconsists of a vascular component in a fibrous stroma. In the most actively growing tumors the vascular component predominates. Vessel walls generally consist of a single endothelial lining directly against the fibrous stroma. This contributes to the capacity for massive bleeding. Deep vessels may have a muscular layer.
Causes of Juvenile Angiofibroma
Common causes of Juvenile Angiofibroma
- Nasal congestion.
- Nosebleeds.
- Capable of eroding bone.
- Invasive.
Symptoms of Juvenile Angiofibroma
Common symptoms of Juvenile Angiofibroma
- Prolonged bleeding.
- Nasal discharge.
- Hearing loss.
- Epistaxis.
- Nasal congestion.
- Rarely anosmia.
- Bulging palate.
- Cheek mass.
Treatment of Juvenile Angiofibroma
Common Treatment of Juvenile Angiofibroma
- Surgical therapy: This remains the mainstay of therapy for stage I-IVa tumors. Several approaches are dependent on the location and extent of the JNA. Options include any combination of lateral rhinotomy and lip split; midface degloving; or transpalatal, transantral, or transzygomatic infratemporal approach.
- Hormonal therapy with diethylstilbestrol before excision has been shown to diminish the vascularity of the JNA but is associated with feminizing side effects. Neither is used routinely; their use is isolated to clinical trials. Doxorubicin and dacarbazine are reserved for recurrences.
- Clotting of the vessels of the tumor may cause the tumor to scar and stop bleeding. Embolization is performed by injecting a substance into the blood vessel to block blood flow through it. Embolization may be adequate by itself to correct the nosebleeds, or it may be followed by surgery to remove the tumor.
- Treatment is required if the angiofibroma is enlarging, obstructing the airway, or causing chronic nosebleeds.
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