Angiofibromas are noncancerous tumors that can form in the nose and nasal passages of mostly young men. The pressure caused by growing tumors can cause chronic congestion or obstruction, frequent and sometimes life-threatening nosebleeds, and discomfort or pain. The condition is rare and often can be cured through minimally invasive surgery.
On this page:
What Is Angiofibroma?
Angiofibroma is a rare benign tumor that can form in the nose and/or nasal cavity, which is the open space that air flows through when you breathe through your nostrils.
The condition almost always affects teenage boys or young adult men, which is why it is also known as juvenile nasopharyngeal angiofibroma. Angiofibromas can also be seen in adult men, although rarely. In extremely rare cases, they have also been observed in women.
While these tumors aren't cancerous, they are highly vascular and can spread to other tissues in the nose. The pressure buildup caused by tumors can cause issues with the sinuses, eyes, skull, or even brain. Severe tumors occur in about 10% to 37% of people with angiofibromas.
Surgery is the primary treatment used to cure angiofibromas. Because they are benign tumors, the prognosis for angiofibromas is favorable.
How common are angiofibromas?
Angiofibromas are rare, accounting for only 0.05% to 0.5% of all head and neck tumors. It occurs anywhere from 1 in 150,000 people to as few as 1 in 1.5 million people.
What causes angiofibromas?
Doctors do not know what causes angiofibromas.
It is thought that they could be related to:
- Genetics.
- Hormonal imbalances.
- Human papillomavirus (HPV).
- Vascular disorders — Involving veins, arteries, and blood vessels.
More research needs to be done to determine what causes these tumors.
Angiofibroma risk factors
- Angiofibroma most often forms in boys between 7 and 25 years of age.
- Young men of Indian and Middle Eastern descent seem to be at the highest risk of developing angiofibromas.
Complications of angiofibroma
Untreated angiofibromas may keep growing, and symptoms will get worse.
People with these tumors may develop more issues, including:
- Arterial vasospasm — Restricted blood vessels.
- Damage to the nerves that run from the brain to the head and neck.
- Double vision or blindness as the tumor damages the nerves responsible for sight or the junction where these nerves meet the brain.
- Hearing loss if the tumor grows into the tube connecting the middle ear to the nasal cavity.
- Infarction — Blocked blood supply.
- Life-threatening nose bleeds.
- Loss of sense of smell.
- Paralysis of parts of the face.
- Uncontrolled eye movement.
Most angiofibromas can be totally removed. But in 33% of advanced angiofibromas, total removal of the tumor may be difficult or impossible. Of these, about 30% to 38% of tumors will recur (come back). If a tumor is going to recur, it will most likely happen within the first three years after surgery.
In people who undergo radiation to further treat angiofibromas, some radiation-induced cancers — such as basal cell and squamous cell carcinomas — have been observed; however, this is very rare.
What Are the Signs and Symptoms of Angiofibroma?
Because of the slow-growing nature of these tumors, people can be asymptomatic (without symptoms) for a long time.
The most common symptoms of angiofibromas are:
- Chronic blockage of the nasal cavity.
- Frequent bloody noses.
- Frequent runny noses.
- Headaches.
- Nausea and vomiting.
- Pain or abnormal sensations.
- Pressure in your ear(s).
- Vision problems.
If the tumor presses on nerves, you may lose vision or feeling in your face.
Some people may also form facial defects, such as:
- Drooping eyelids.
- Proptosis — Bulging eyes.
- Swelling of the cheek(s).
When should I see a doctor about angiofibromas?
Talk to your doctor if you have been experiencing chronic obstruction or congestion of one or both of your nasal passages that hasn't improved with allergy medications. Also report frequent and abrupt nosebleeds, headaches, or excessively runny noses, especially if you've had these symptoms for six months or more.
How Do You Diagnose Angiofibroma?
Angiofibromas are most commonly diagnosed in boys between the ages of 10 and 19, but they have been seen in boys as young as 7 and as old as 25.
Your doctor will conduct a physical exam to look for flesh-colored to pink or reddish-purple, firm, dome-shaped bumps or papules inside the nasal cavity. They may also order imaging, such as an MRI or a CT scan of your head and facial bones, to confirm a diagnosis. Imaging also helps your doctor see the size of the tumor, where it has grown, and what other structures it is impacting.
These tumors often have many blood vessels and are not surrounded by muscle. For this reason, doctors generally avoid taking a biopsy (a sample of tumor tissue) because it could lead to extensive bleeding.
How Do You Treat Angiofibroma?
Hormone therapy
Hormone therapy is a new type of treatment for angiofibroma. The drug flutamide is a hormone therapy that blocks proteins that bind to the male sex hormone testosterone. This drug seems to help shrink tumors before surgery, but it is not a cure for angiofibroma on its own. Doctors are currently testing it in clinical trials.
Minimally invasive surgery
The most common treatment for angiofibromas is surgery. The success rate for surgical removal of angiofibromas is high; most people are cured through surgery. The greatest risk associated with surgery is excessive bleeding due to the vascular nature of these tumors.
UPMC surgeons prefer to treat angiofibromas directly using the endoscopic endonasal approach (EEA), an innovative minimally invasive surgery that was pioneered and refined at UPMC. We are among the most prolific providers of this procedure worldwide.
This state-of-the-art, minimally invasive approach allows surgeons to access the tumor through the natural corridor of the nose. Most angiofibromas can be removed without an open incision, but larger tumors may require a hidden incision under the upper lip or, rarely, next to the eye. Because EEA typically involves no incision, there is minimal to no scarring, and recovery time is faster than that of traditional surgery.
What happens during the surgery?
Before surgery, your doctor may perform an imaging test and a procedure called an angiogram. This test uses X-rays and a special dye to highlight blood vessels and determine the extent of the tumor’s vascular connection.
With the blood vessels highlighted, your doctor can embolize (cut off) the blood going to the tumor. Embolization helps prevent blood loss when the surgeon removes the growth. Your doctor will then remove the angiofibroma through your nose and nasal cavities.
Because these tumors are most likely to recur within the first three years after surgery, your health team will want to monitor your progress once or twice a year for at least four years.
Radiation
In certain instances, radiation may be needed and can be started soon after surgery. Radiation is generally avoided as a primary treatment for these tumors, though it may be used in addition to surgery in certain cases.
Small tumor remnants should be monitored using a watch-and-wait approach, but often do not cause problems after puberty is complete. On rare occasions, your doctor may suggest radiation exposure to kill any tumor cells left in the nasal or skull cavity.
Angiofibroma tumors that may need radiation include those that:
- Come back after repeat surgery.
- Have extended into the skull.
- Surgeons can't remove safely.
