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What

Juvenile angiofibroma, also called juvenile nasopharyngeal angiofibroma (JNA), is a rare noncancerous growth made of tissue fibers and blood vessels. The tumor usually first appears in the back part of the nose and may be visible. Though a juvenile angiofibroma does not spread to other areas of the body, it can be aggressive and grow into surrounding parts of the face. This tumor can even invade into and break down bone. The tumor can grow in all directions that can cause the center part of the nose (nasal septum) to be moved out of the way. The tumor can also grow into the sinuses in the boney part of the face. The tumor itself is composed of numerous blood vessels with thin walls that are missing the normally present smooth muscle layer. The smooth muscle layer gives blood vessels strength and without it these tumors can bleed easily and when they do bleed, a lot of blood can be lost.

Who

For reasons that are not clear JNA occurs almost exclusively in adolescent males. Symptoms usually begin between the ages of 7 and 21 years, with cases in young childhood and middle age being extremely rare. If a diagnosis of JNA is made in a female, genetic studies and review of the medical information may be necessary because the diagnosis may be incorrect. JNA is very rare and only accounts for 0.05% of all head and neck tumors.

Signs and Symptoms

People with JNA may have several or all of the following signs and symptom: 1. Progressive blockage of the nose 2. Nasal congestion which may cause difficulty breathing 3. Nasal discharge (rhinorrhea), often only on one side 4. Nose bleeds (epistaxis): frequent, recurrent and usually on one side 5. Headaches 6. Facial swelling, inflammation of the sinuses (sinusitis) 7. Hearing loss or ear infections due to blockage of the Eustachian tube (which connects the ear to the throat and allows drainage of fluid) 8. Protrusion of the eye (proptosis or exophthalmos)

Possible Causes

The cause of JNA lesions remains unknown. Researchers believe that they may arise when the connective tissue that covers the inside of the nose and throat is irritated by abnormal blood vessels that are present at birth. Since JNA develops in males, around the time of puberty, hormonal involvement is suspected.

Diagnosis

Adolescent-aged boys typically will come to the doctor with a growth in their nose and have a history of recurrent nosebleeds that occur for no reason. Life-threatening bleeding has occurred when a piece of the mass is cut out for study so the diagnosis is usually made from imaging studies. Imaging studies can also determine the best course of treatment. CT scan and MRI can help show exactly where the tumor is located and define the parts that are involved. Studies of the blood vessels located in the neck (carotid angiography) may be used to determine the best type of surgery and to stop some of the blood flow to the mass before surgery (embolization).

Treatment

Common treatment for JNA is surgical removal. Procedures are done to try and stop some of the blood flow to the tumor before surgery is done. This makes the surgery much safer. Radiation therapy can be used when the tumor cannot be removed by surgery or if the tumor returns after surgery. Radiation therapy is not commonly used for other cases because of the side effects of this treatment. Chemotherapy may be used for recurrent tumors that have grown into surrounding parts of the face.

Prognosis

Without treatment, JNA may grow and affect the surrounding parts of the face. It is thought that these tumors may sometimes go away by themselves, but because there can be a lot of bleeding, that can actually be life-threatening, patients should seek treatment. After successful removal by surgery, the person is cured. However, these tumors often return so the person needs to remain under the care of a physician for monitoring. Recurrent tumors also require treatment.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Medline Plus http://www.nlm.nih.gov/medlineplus/ency/article/001572.htm A nice summary of the disease with good definitions of medical terminology.

Google Search for Juvenile Angiofibroma

References and Sources

1. Juvenile Nasopharyngeal Angiofibroma. www.emedicine.com/ent/topic470.htm 2. Juvenile Nasopharyngeal Angiofibroma. www.utmb.edu/oto/Grand_Rounds_Earlier.dir/JNA_Fibroma_1993.txt 3. Angiofibroma. www.amershamhealth.com/medcyclopaedia/Volume%20VI%202/angiofibroma.asp 4. Stedman’s Medical Dictionary, 27th ed. Lippincott Williams & Wilkins ©2000.