Juvenile angiofibromas are benign vascular tumors that usually develop in the nasopharyngeal area in young males around the time of puberty.
The term (juvenile angiofibroma) has been applied because this head and neck tumor typically develops in young males at puberty. However, in up to 20% of this head and neck tumor are diagnosed after the age of 20. Juvenile angiofibromas have been reported up to the age of 50.
Almost all patients that have a juvenile angiofibroma are males. Few reports exist in females. The differential diagnosis include angiomatous polyps and not true angiofibromas.
There is a higher incidence of angiofibromas in Egypt, India, Southeast Asia, Kenya, and Mexico.
There has been some relationship between the behavior of angiofibromas and sex hormones. These include:
- Reduction in size of angiofibromas is observed after exogenous estrogen therapy.
- Increase in size occurs in some angiofibromas following testosterone treatment.
- The result of neurological testing in patients with juvenile angiofibromas of the nasopharyngeal region had shown normal pituitary-gonadal axis.
The symptomatology of juvenile angiofibromas is related to the size and direction of extension in the growth. The most typical presentation are nasal obstruction and nasal bleeding or epistaxis. The usual first symptom of angiofibromas is the appearance of nasal obstruction with secondary sinusitis due to obstruction of the drainage of the sinuses. This may be associated with some decrease in hearing due to longstanding otitis.
The second most frequent symptom of juvenile angiofibromas is recurrent and repeated hemorrhage that may be severe.
Clinical examination of the nasal and nasopharyngeal areas reveals a reddish raised tumor belonging to the nasopharynx which is typical of angiofibromas. The angiofibromas may bleed on contact. There is usually no ulceration on the surrounding areas. Biopsies in angiofibromas should be avoided based on the clinical history as the bleeding can be quite severe.
It is believed that juvenile angiofibromas will continue to grow up to the late teens and early 20's where there may be some spontaneous regression. However, this is controversial and most of these patients require treatment.
Computed tomography is a very useful image modality to see the presence of juvenile angiofibromas, their extension, and any involvement of bony structures.
Magnetic resonance imaging of juvenile angiofibromas has become the prime modality for assessment of tumor vascularity and tumor extent, however, it is less accurate than CT for bony landmarks.
Angiography is mandatory in the great majority of patient's with juvenile angiofibromas. Angiography in juvenile angiofibromas would be used to determine the blood supply to the tumor and will determine the extension of the tumor and blood supply for this head and neck nasopharyngeal tumor from the external carotid and internal carotid arteries. In extensive tumors, there may be extension towards the vertebrobasilar system.
Embolization is a technique of occlusion of abnormal blood vessel by means of angiographic catheters at the time of the angiographic examination in juvenile angiofibromas.
Embolization of juvenile angiofibromas has become essential in the proper management of this nasopharyngeal tumors. Juvenile angiofibromas is one of the most vascular tumors of the maxillofacial region. Therefore, surgical excision is significantly facilitated by embolization prior to surgical removal of this tumor.
Palliative embolization means the use of embolization with more aggressive embolic materials in patients that are beyond the ability to surgically remove them. Palliative embolization may be beneficial in controlling the bleeding of this tumors. It may be also effective in preventing further growth and may be also of use in stabilizing these tumors. Palliative embolization of juvenile angiofibromas is reserved for those patients in whom conventional surgical intervention is not possible for either anatomical or medical reasons.
Surgery of angiofibromas requires preoperative embolization of the head and neck nasopharyngeal tumor. Surgical excision is the best treatment in controlling this disease. The use of preoperative embolization of juvenile angiofibromas has made surgical excision of this otherwise benign tumor markedly improved.
The use of radiation therapy is reserved for tumors that cannot be surgically excised. And if used, it should be done prior to embolization. A good combination may be radiation followed by devascularization using embolization of the juvenile angiofibroma.
Other nasopharyngeal vascular tumors include paragangliomas, meningiomas, neuroblastomas, esthesioneuroepitheliomas, hemangiopericytomas, hemangioendotheliomas, hemangiopericytoma-like tumors, and extracranial meningiomas. Less frequent, metastatic kidney tumors and metastatic thyroid tumors.
