Nasopharyngeal angiofibroma: Fatal proof Surgery

It came as a big shock for Arvind and Sunita when they heard about their 13-year-old son Ashish suffering from Nasopharyngeal Angiofibroma. the name itself was very scary to digest. the first reaction from both of them was is it something serious or life-threatening and how much time it will take their son to recover. they just remembered how Ashish complained of nose bleeding and difficulty in breathing and they immediately rush to the nearest hospital.

Doctor suggested immediate operation and removal of tumor. but on another hand also portrayed it as a life-threatening process as the tumor receives a heavy blood supply and there are high chances of death due to blood loss during surgery.
parents who are already in a high tensed state not able to decide anything. after some time a senior doctor who came up for a routine check-up listened to the case of their son and asked both of them to opt for interventional Radiology method. as the method can be less fatal for their child's health and will remove the tumor safely as well.

The parents immediately agreed to the doctor's suggestion and asked for an expert help
senior doctor refers them to one of the experts in the field of Interventional Radiology, Dr. Atul Rewatkar & Dr. Rajesh Mundhada who is highly experienced and dedicatedly working in the field of Interventional Radiology with numerous life-saving Treatments.

Ashish has been admitted on the immediate basis and after a day-long treatment and examination, the surgery for tumor removal was safe and successful. the parents were very happy and thankful to the doctors for saving their child's life without any danger.
but on a similar note, they have many questions in their mind related to the disease and the treatments which have been properly explained to them by Doctors.

Juvenile Nasopharyngeal Angiofibroma

Juvenile nasopharyngeal angiofibroma (JNA) is a benign vascular tumor that appears in the nasal cavity. Although it is non-malignant (not cancerous), it can expand quickly and extensively. JNA can spread into a variety of compartments in the head – including the sinuses, the orbit (where the eye is located), and the brain – which can make it very difficult to treat.

• JNA generally affects adolescent boys; very infrequently it presents in younger children or post-adolescent males; it is rarely, if ever, seen in a female.
• Typically, once patients with JNA reach their early 20s, the disease mysteriously begins to resolve. Rarely, in some patients, the disease will continue into their 30s.
• Surgery is the principal treatment. In cases where the entire tumor cannot be surgically removed, patients may receive medications to minimize tumor growth until the disease resolves in early adulthood.

What are the symptoms of JNA?

In the early stages of the disease, patients with juvenile nasopharyngeal angiofibroma might not show any symptoms. As the tumor expands, however, the following symptoms may develop:

• Difficulty breathing through the nose (due to airway obstruction)
• Nosebleeds
• Headaches
• Double vision and other cranial nerve signs or symptoms if the tumor has expanded significantly Patients rarely feel any significant pain.

What causes juvenile nasopharyngeal angiofibroma?

Scientists do not fully understand what causes JNA. Although it is not hereditary, children with family members who have familial adenomatous polyposis are slightly more likely to have JNA. Because the disease presents predominantly in adolescent boys, some studies have explored hormonal influences in JNA, but these studies have not been conclusive.

What are the treatment options for juvenile nasopharyngeal angiofibroma?

First line therapy is usually surgery, with the goal of removing as much of the tumor as possible. Ideally, the entire tumor is removed. In some situations, depending on where the tumor has spread, complete removal may not be possible.

The initial treatment of JNA has two components:

• Angiographic embolization by interventional radiologists; this is used immediately before surgery to close off the blood vessels that supply the tumor in order to limit blood loss during surgery.
• Surgery to remove the tumor. This surgery is often done endoscopically – minimally invasive surgery performed through the nose guided by a special telescope (endoscope) and your child’s pre-operative imaging studies. If the tumor is localized (not spread far beyond the nasal cavity), this can be accomplished in one surgery. However, if the tumor has expanded significantly, multiple surgeries may be required. If it has impacted the brain, neurosurgeons will also be involved. Surgery for JNA is very complex due to where the tumor is located and should be done by a surgeon with specific expertise in this type of surgery.

Sometimes surgeons will not be able to remove the entire tumor, such as when the tumor has expanded into the cranial cavity and is impacting the brain or carotid artery. In such instances, remnants (pieces) of tumor tissue will remain. Because it’s possible for these tumor remnants to regrow, your treatment team will discuss additional therapies to treat this residual disease with you and your child.

This additional therapy may include angiogenesis inhibitors – medications that inhibit the growth of new blood vessels. Sometimes, these medications can shrink the JNA tumor sufficiently to allow surgeons to further resect (surgically remove) the tumor. Other times, the patient will need to continue to take the medications until the disease resolves in early adulthood.

Radiation therapy is very rarely considered, as radiation, particularly so close to the brain, can have long-term side effects. Medications can often control the disease with fewer side effects than radiation.