A rare, benign, but locally aggressive vascular tumor that develops in the nasopharynx, juvenile nasopharyngeal angiofibroma (JNA) mostly affects teenage boys. By destroying nearby tissues and structures, it can act aggressively even though it is histologically benign (non-cancerous). It is essential for patients and physicians to comprehend the nature of JNA, its presentation, diagnosis, and available treatments, including cutting-edge methods like Gamma Knife Radiosurgery.
What is it?
Juvenile Nasopharyngeal AngiofibromaThe fibrovascular tumor known as JNA typically begins close to the sphenopalatine foramen, which is situated at the rear of the nasal cavity. Despite being benign, the tumor is well-known for its propensity to bleed heavily, both naturally and during surgery, because of its abundant blood supply.
The study of epidemiology
The study of epidemiology_ Juvenile Nasopharyngeal Angiofibroma
Age group: Boys between the ages of 10 and 25 are most frequently observed.
Gender: It almost primarily impacts men.
Less than 0.05% of all head and neck tumors are caused by this tumor.
Causes and Symptoms
Early signs and symptoms include:Causes and Symptoms_ Juvenile Nasopharyngeal Angiofibroma
Nosebleeds that are severe or frequent
Congestion of the nose
Runny nose
As the tumor grows, the symptoms worsen. More severe symptoms may include:
Swelling in your face, particularly in your cheeks
The reasons for JNA are not entirely understood by experts. Hormones are probably involved because these tumors almost exclusively affect men. According to experts, JNA tumors are not inherited.
Risk factors for juvenile nasopharyngeal angiofibroma
For JNA, there aren’t many distinct risk factors. Although JNA is not inherited, you are at a higher risk of developing it if any members of your biological family have familial adenomatous polyposis (FAP), a rare disorder in which precancerous polyps develop in your large intestine. This relationship is still being investigated by experts.
What side effects might juvenile nasopharyngeal angiofibroma cause?
If untreated, can result in:
Having trouble breathing
Speech, hearing, and vision problems
Deformities of the face, such drooping eyelids or protruding eyes
Anosmia is the loss of smell
Severe blood loss and nosebleeds
Shift in mental condition
How is angiofibroma of the juvenile nasopharynx diagnosed?
During an examination, a medical professional can make the diagnosis of JNA. They will examine your nose and then enquire about your symptoms. For additional testing, they can also recommend that you see an otolaryngologist (ENT).
Which diagnostic procedures can be used to identify juvenile nasopharyngeal angiofibroma?
Your doctor will want to perform imaging testing if they suspect JNA. These could consist of:
Computed tomography, or CT, scans.
Magnetic resonance imaging, or MRI.
Scans using PET (positron emission tomography).
To examine the tumor, they might also undertake a nasal endoscopy. A light and camera are attached at the end of an endoscope, which is a long, flexible tube. To view within your nasal cavity, a healthcare professional will carefully insert an endoscope into your nose. You should not experience discomfort since they will apply a numbing spray, which will cause pressure.
What is the course of treatment for juvenile nasopharyngeal angiofibroma?
The recommended treatment for juvenile nasopharyngeal angiofibroma is surgery. Radiation therapy is also necessary for some people.
The location and size of the JNA tumor determine the course of treatment.
Surgery for nasopharyngeal angiofibroma in childrenThe entire JNA tumor is intended to be removed during surgery. A surgeon may be able to remove the tumor through your nose (endoscopic surgery), depending on its size and location. This procedure will be carried out in a hospital by an otolaryngologist.
Radiation treatment for JNA
Because of its placement, surgeons may not be able to remove the entire tumor in some circumstances. Your doctor might suggest radiation treatment or additional surgery if the tumor cannot be removed or if it returns after surgery. High-energy X-ray beams are used in this treatment to reduce the tumor’s size.For prevention, there is no known way to prevent JNA tumors.
Gamma-Knife Radiosurgery for Nasopharyngeal Angiofibroma in Children
A non-invasive, extremely accurate type of radiation therapy called Gamma Knife Radiosurgery (GKRS) targets the tumor with concentrated radiation while preserving nearby healthy tissues. It is a promising complement or substitute for surgery in the treatment of JNA, especially when the tumor has intracranial extension or surgery is not practical.
Gamma Knife Indications in JNA:
Remaining tumor after surgery
Recurrence following surgery
Tumors involving the deep base of the skull or the brain
Individuals who are not good candidates for surgery
Gamma Knife Radiosurgery Benefits:
Minimally invasive—no incision during surgery is necessary
Targeting the tumor with high precision while preserving important surrounding structures
Reduced likelihood of problems
An outpatient surgery that requires little recovery time
Outstanding rates of tumor control in long-term monitoring studies
Results:Research shows tumor control rates of between 85 and 90 percent, with tumors gradually regressing over several months. When surgery is not enough to treat symptoms like nasal blockage and epistaxis, GKRS has been proven successful.
Diagnosis and Monitoring
The majority of patients have outstanding long-term results when they receive the right care. Monitoring for recurrence requires routine imaging follow-up, particularly in the initial years after therapy. MRI scans are advised periodically for patients receiving Gamma Knife treatment in order to evaluate tumor regression.
In conclusion
A difficult but treatable disorder is juvenile nasopharyngeal angiofibroma. Although surgical resection is still the mainstay of treatment, Gamma Knife Radiosurgery has become a useful method for treating inoperable or residual tumors because it provides great control with little morbidity.To create the optimal treatment plan for every patient, a multidisciplinary team of radiation oncologists, neurosurgeons, ENT specialists, and interventional radiologists is required.Source:
