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​Angiofibroma (Juvenile Nasopharyngeal Angiofibroma)

What is Angiofibroma?

Angiofibroma is a benign (noncancerous) nasal cavity tumor that almost exclusively affects adolescent boys. It also may be referred to as juvenile nasopharyngeal angiofibroma (JNA).

The age range for this disease is 7 to 19 years old, with most people being diagnosed between 10 and 19 years old.

Juvenile nasopharyngeal angiofibroma is the most common benign tumor of the nasopharynx, but has a relatively low incidence. It accounts for only 0.5 percent of all head and neck tumors.

Though benign, it often acts in a malignant manner by eroding into the surrounding sinuses, orbit, or cranial vault.

At UPMC, the preferred surgical treatment for angiofibroma is the Endoscopic Endonasal Approach (EEA). This innovative, minimally invasive technique uses the nose and nasal cavities as natural corridors to access hard-to-reach or previously inoperable tumors.

Benefits of EEA include:

  • No incisions to heal
  • No disfigurement
  • Faster recovery time

EEA has proven safe and effective in removing angiofibromas from children. More than 100 children have been treated by UPMC skull base surgeons using EEA — more than at any other neurosurgery center in the world.

Diagnosing Angiofibroma

To diagnose angiofibroma, the doctor will perform a physical exam and ask about any symptoms.

Angiofibroma symptoms

The most common symptoms of angiofibroma include:

  • Nasal obstruction
  • Nose bleeding (epistaxis)
  • Runny nose (rhinorrhea)

Some people also may develop facial deformities or abnormalities, including:

  • Cheek swelling
  • Drooping eyelids
  • Bulging eyes
  • Cranial nerve palsies

Complications of angiofibroma

  • Hearing loss may result from obstruction of the Eustachian tube.
  • Double vision or blindness may result from pressure on the optic nerves and chiasm if there is erosion into the cranial cavity.
  • Rarely, people with juvenile nasopharyngeal angiofibroma may suffer anosmia — the loss of the sense of smell.

Tests for diagnosing angiofibroma

On physical exam, a pale, smooth mass may be visible inside the nasopharynx.

Because the tumor is composed of blood vessels without a muscular coat, a biopsy could lead to extensive bleeding and doctors generally do not perform one.

An MRI or CT scan of the head and facial bones confirms the clinical diagnosis of angiofibroma and shows the extension of the tumor.

Angiofibroma Treatments

Surgery

The most common treatment for angiofibroma is surgery.

Angiofibromas may be approached directly using the Endoscopic Endonasal Approach (EEA). This state-of-the-art, minimally invasive approach allows surgeons to access the tumor through the natural corridor of the nose, without making an open incision. Surgeons then remove the angiofibroma through the nose and nasal cavities.

EEA offers the benefits of no incisions to heal, no disfigurement, and a faster recovery time.

If you need complementary treatments, such as radiation, those therapies can begin soon after EEA surgery.

Prior to surgery, an angiogram may be performed. This imaging test uses x-rays to view blood vessels that have been injected with a contrasting dye, to allow for embolization of the tumor.

Embolization involves cutting off the blood supply to the tumor. It significantly reduces blood loss during surgery and tumor removal.

Radiation therapy

Radiation therapy may be used for people with angiofibroma tumors that have extended into the cranial cavity, can't be reached safely by surgery, or have returned after previous treatment.

Chemotherapy

Hormonal flutamide, which blocks testosterone receptors, has been shown to reduce tumor size effectively.

The use of this medication is restricted to those enrolled in clinical trials.

 

Meet Matthew

Matthew Carls – Angiofibroma Patient Story 

UPMC surgeons performed an extraordinary, nine-hour surgery that allowed this average 10-year-old to return to the everyday fun of being a kid.

Read more »

 
 

Meet Baylor

Baylor Cox – Angiofibroma patient story 

This high school football player and his family turned to the experts at UPMC when a rare and large tumor began pushing into his nasal cavity.

Read more »

 

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