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Juvenile Angiofibroma

Juvenile angiofibroma - an uncommon disorder - is a highly vascular, nasopharyngeal tumor made up of masses of fibrous tissue containing many thin-walled blood vessels. These tumors are found primarily in adolescent males and are extremely rare in females. Incidence is higher in Egypt, India, Southeast Asia, and Kenya than in the United States and Europe. The prognosis is good with treatment.

Causes

Juvenile angiofibroma is relatively uncommon. It typically is discovered in adolescent boys and is associated with nasal congestion, a visible nasal mass, and nosebleeds. The tumor is locally invasive and capable of eroding bone.

Signs and Symptoms

Usually between ages 7 and 21 , the patient reports a history of unilateral or bilateral nasal obstruction and severe recurrent epistaxis. The patient also may reveal a history of purulent rhinorrhea and serous otitis media, with resultant hearing loss from eustachian tube obstruction. Inspection may reveal facial deformity and nasal speech.

Diagnostic tests

Examination with a nasopharyngeal mirror or nasal speculum permits visualization of the tumor, which appears as a blue mass in the nose or nasopharynx, and X-rays show bowing of the posterior wall of the maxillary sinus.

Angiography is used to determine the size and location of the tumor and source of vascularization. A computed tomography scan allows visualization of vascular mass.

Biopsy is contraindicated because of the danger of hemorrhage.

Treatment

Several surgical methods, ranging from avulsion to cryosurgical techniques, are used to treat juvenile angiofibroma. Whichever surgical method is used, the tumor must be removed in its entirety and not in pieces. Embolization with a substance to occlude the vessels may decrease the potential for bleeding. Excision is usually rescheduled 12 days after embolization to allow inflammation to decrease. Surgical excision is preferred after embolization with Teflon or an absorbable gelatin sponge to decrease vascularization. Blood transfusions may be necessary during avulsion. Preoperative hormonal therapy may decrease the tumor's size and vascularity.

Although radiation therapy produces only a temporary regression in an angiofibroma, it remains the treatment of choice if the tumor has expanded into the cranium or ocular orbit. Because the tumor is multilobular and locally invasive, recurrent symptoms are common (occurring in about 30% of patients) during the first year after treatment but are uncommon after 2 years.

Prevention

There is no known way to prevent this condition.



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