Introduction:
Juvenile nasopharyngeal angiofibroma mostly comprises the blood vessels. These tumors are not cancerous but can be very aggressive and severe and can potentially extend from the nasal cavity into the sinuses, eye sockets, skull, and finally, the brain. This condition is also called juvenile angiofibroma. It is an extremely uncommon condition, with only 50 new cases annually in the United States. It constitutes about 0.5 % of all head and neck tumors that occur in 1 in 1,50,000 individuals. Recent reports show that the tumor is more prevalent in the Indian subcontinent than in the West.
Who Is Affected by Juvenile Nasopharyngeal Angiofibroma?
Juvenile nasopharyngeal angiofibroma commonly affects adolescent and prepubescent boys. Boys between the ages of 10 and 20 are commonly affected. In rare cases, older men or boys as young as seven years old may be affected. Females are least likely to be affected by juvenile nasopharyngeal angiofibroma.
There are not many precise risk factors for this condition. Juvenile nasopharyngeal angiofibroma is not a hereditary condition, but one is likely to develop this condition if any of their biological family members have familial adenomatous polyposis (an uncommon condition in which precancerous or benign polyps develop in the large intestine). Studies are in progress to understand the relationship between the two.
What Anatomical Landmarks Are Involved in Juvenile Nasopharyngeal Angiofibroma?
Juvenile nasopharyngeal angiofibroma initially develops in the lateral wall of the nasal cavity, near the superior border of the sphenopalatine foramen. This growth originates in the submucosa of the floor of the nasopharynx and progresses to the nasal septum and the posterior space of the nose resulting in airway obstruction.
Extensive growth involves the nasal fossa, sphenoidal sinus, middle turbinate, pterygomaxillary fossa, and posterior wall of the maxillary sinus. The tumor may also extend to the infratemporal and middle cranial fossa.
What Are the Causes of Juvenile Nasopharyngeal Angiofibroma?
The causes of juvenile nasopharyngeal angiofibroma have not been fully understood. Since these tumors mostly occur in individuals assigned males at birth, hormones are likely to contribute to the occurrence of juvenile nasopharyngeal angiofibroma. Experts do not believe in the fact that these tumors are hereditary. It is believed that the cells that develop into these tumors are left over from early pregnancy.
What Are the Signs and Symptoms of Juvenile Nasopharyngeal Angiofibroma?
The symptoms in the early stages are as follows:
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Runny nose.
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Severe or frequent bleeding from the nose (the bleeding is usually from one nostril).
Symptoms can worsen as the condition progresses. Symptoms in the advanced stages include:
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Swelling in the face, especially in the cheeks.
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Pain in the upper regions of the face.
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Headaches.
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Loss of vision.
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Double vision.
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Bulging eyes and drooping eyelids.
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Hearing loss.
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Facial numbness.
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Sleep apnea (a sleep disorder in which breathing stops and starts repeatedly).
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Speech issues.
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Difficulty breathing through the nose as a result of airway obstruction.
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Difficulty smelling odors.
How Can Juvenile Nasopharyngeal Angiofibroma Be Diagnosed?
A healthcare professional will initially inquire about the symptoms and examine the nose. If required, one might be referred to an otolaryngologist (an expert who treats the head and neck problems). The following imaging tests may be suggested:
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CT (Computed Tomography) Scans: A combination of computer technology and X-rays are used to produce horizontal and vertical slices or cross-sectional images of a particular area. CT scans provide clear details of the bony structures in the body.
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Magnetic Resonance Imaging (MRI) Scans: In this diagnostic procedure, a large magnet, radio waves, and a computer are used to produce two and three-dimensional pictures of the soft tissue organs in the body.
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PET (positron emission tomography) scans.
Nasal endoscopy may also be performed to view the tumor using a thin, flexible, or rigid tube containing a tiny light and a camera attached at the end. A numbing spray will be used before inserting the endoscope so that one feels only the pressure and not the pain. Rarely, a biopsy may be performed to confirm the diagnosis. But, a biopsy is not recommended since these tumors are vascular (contain blood vessels). Imaging techniques are sufficient to arrive at a diagnosis.
How Can Juvenile Nasopharyngeal Angiofibroma Be Treated?
Surgery is the treatment of choice for treating juvenile nasopharyngeal angiofibroma. A few individuals may also require radiation therapy. Treatment depends on the size and location of the tumor. The treatment modalities are as follows:
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Angiographic Embolization: This is done immediately before surgery to shut the blood vessels that supply the tumor to reduce blood loss. This is carried out by interventional radiologists.
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Juvenile Nasopharyngeal Angiofibroma Surgery: The surgery aims to remove the tumor as a whole. Depending on the size and location of the juvenile nasopharyngeal angiofibroma, a surgeon will remove the tumor through the nose through a procedure called endoscopic surgery. This surgery is performed in a hospital setting. Tumors that have expanded significantly might require multiple surgeries.
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Radiation Therapy: At times, due to the delicate position of the tumor, surgeons will be unable to remove the entire tumor. In such cases or cases of recurrence, the surgeons may perform radiation therapy or repeat surgery. Radiation therapy involves the use of high-energy X-ray beams to destroy the tumor. However, radiation therapy is least preferred since it can have long-term side effects as it involves regions close to the brain.
Instead, angiogenesis inhibitors or medications that hinder the growth of new blood vessels may be prescribed. Sometimes, these medications will effectively shrink the tumor, which enables the surgeons to further remove the tumor. In other cases, the individual needs to continue taking the medications until the condition resolves in early adulthood.
Is Follow-Up Treatment Required for Juvenile Nasopharyngeal Angiofibroma?
Even if the tumor is entirely removed during surgery, there are chances of recurrence. Because of this, individuals with juvenile nasopharyngeal angiofibroma might require an annual follow-up, during which the physician will carry out endoscopic examinations and take new imaging scans to check for any recurrence.
If the tumor cannot be removed completely, then more frequent visits will be required to evaluate the blood work, assess the side effects, and adjust the dosages of the medications.
What Are the Complications Associated With Juvenile Nasopharyngeal Angiofibroma?
If juvenile nasopharyngeal angiofibroma is not treated, it can lead to the following complications:
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Difficulty breathing.
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Alteration in the mental status.
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Vision, hearing, and speech-related issues.
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Severe bleeding from the nose and massive loss of blood.
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Anosmia (loss of sense of smell).
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Facial abnormalities like bulging eyes or drooping eyelids.
Conclusion:
Juvenile nasopharyngeal angiofibroma is a rare, noncancerous condition that involves the nasal cavity. There are no ways to prevent the occurrence of this tumor. Prompt treatment is the only option to treat this condition. The prognosis of this tumor depends on the size and location of the tumor. In most cases, the tumor is removed through a surgical procedure. Following surgery, follow-up appointments may be scheduled to evaluate and check for any recurrence. Usually, recurrence occurs within two years following surgery. Recurrent cases may require another surgery or radiation therapy.
