What are the other Names for this Condition? (Also known as/Synonyms)

• JNA (Juvenile Nasopharyngeal Angiofibroma)
• Juvenile Angiofibroma
• Nasal Juvenile Angiofibroma

What is Juvenile Nasopharyngeal Angiofibroma? (Definition/Background Information)

• Juvenile Nasopharyngeal Angiofibroma (JNA) is an uncommon, benign tumor of the nose that is generally seen in boys. The tumor is locally aggressive and can invade into surrounding structures and cause significant complications
• There are no clear risk factors identified for the tumor, but it is seen during puberty. It is believed that the cause of Juvenile Nasopharyngeal Angiofibroma is hormonal factors
• The signs and symptoms associated with Juvenile Nasopharyngeal Angiofibroma include nosebleeds, stuffy nose, and headaches. If the tumor spreads to adjoining regions in the face or mouth, it can cause reduced sense of smell, deafness, or even facial disfigurement
• Occasionally, Juvenile Angiofibroma can invade into brain tissue and cause neurological signs and symptoms. Also, in some cases, severe bleeding from the tumor can result in anemia with associated symptoms
• The treatment of Juvenile Nasopharyngeal Angiofibroma may include arterial embolization, radiation therapy, or a complete surgical excision and removal depending on the assessment by the healthcare provider
• The prognosis of Juvenile Nasopharyngeal Angiofibroma is generally good, if the tumor can be successfully and completely removed. The prognosis may be affected by complications, or if a malignant transformation of JNA takes place (which can occur in rare cases)

Who gets Juvenile Nasopharyngeal Angiofibroma? (Age and Sex Distribution)

• Juvenile Nasopharyngeal Angiofibroma is a rare tumor. The tumor is considered rare, because only 0.05% of all head and neck tumors are Juvenile Angiofibromas
• JNA is almost exclusively seen in males (adolescent boys) in the age range 7-19 years. The tumor is hardly known to occur in females
• Individuals of all racial and ethnic background may be affected; no geographical preference is also observed

What are the Risk Factors for Juvenile Nasopharyngeal Angiofibroma? (Predisposing Factors)

• The risk factors for Juvenile Nasopharyngeal Angiofibroma are generally unknown or unidentified
• However, the condition is mostly observed in young boys in the pubertal age

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Juvenile Nasopharyngeal Angiofibroma? (Etiology)

• The cause of development of Juvenile Nasopharyngeal Angiofibroma is identified with hormonal influence, such as due to androgen hormones
• Since, there is a hormonal surge during puberty in boys, it supports the theory of hormonal influence as a causative factor for this tumor growth and development

What are the Signs and Symptoms of Juvenile Nasopharyngeal Angiofibroma?

The signs and symptoms of Juvenile Nasopharyngeal Angiofibroma include:

• Presence of benign tumors of size between 0.5 to 5 cm, generally located in the nose (nasal cavity or nasopharyngeal region)
• Since the tumors are present in the nose, they may cause:
  • Difficulty in breathing
  • Nosebleeds
  • Headaches
• Some tumors are aggressive and invade locally into adjoining tissue, cartilage, and bones
• If the tumor invades into surrounding structures affecting their function, then the following symptoms may be seen:
  • Decreased smell or loss of smell
  • Deafness, ear aches
  • Deformity of the face

How is Juvenile Nasopharyngeal Angiofibroma Diagnosed?

The following are the diagnostic methods to help identify Juvenile Nasopharyngeal Angiofibroma:

• A thorough physical examination and a complete medical history are very crucial
• Radiological studies of the head and neck region that include X-rays, CT or MRI scans
• Vascular angiography study: If such a study is undertaken, then the findings of the study is helpful to a surgeon during removal of the tumor, in order to help minimize bleeding
• Complete excision of the tumor: The excised tumor is sent to a laboratory for a pathological examination. The pathologist examines the tumor under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Word of caution: The tumor should not be partially biopsied, because it can cause significant bleeding during the process. Hence, it must be completely removed as part of the biopsy.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Juvenile Nasopharyngeal Angiofibroma?

There may be significant complications due to Juvenile Nasopharyngeal Angiofibroma that may include:

• Some bleeding can be prolonged and heavy
• Severe, prolonged bleeding can cause anemia, due to which the individuals may have pale skin, tiredness, shortness of breath, and rapid heartbeat
• Occasionally, the tumor can invade into brain tissue causing neurological signs and symptoms
• Severe stress and anxiety issues due to cosmetic concerns (facial deformity)
• Recurrence of JNA following its incomplete removal
• In rare cases, these tumors can become malignant; such transformation can affect the prognosis of the condition

How is Juvenile Nasopharyngeal Angiofibroma Treated?

The treatment options for Juvenile Nasopharyngeal Angiofibroma may include the following procedures and measures:

• Arterial embolization of Nasal Juvenile Angiofibroma is a possible treatment option. Here the blood supply to the tumor is blocked resulting in tumor death
• Hormonal therapy to decrease size of the tumor
• If the tumor is locally-invasive, then radiation therapy may be proposed. There are 2 types of radiation therapies that the healthcare provider may recommend:
  • Stereotactic radiation therapy: The radiation is delivered to the tumor using an external device or equipment
  • Conformal radiation therapy: It is a 3-dimensional procedure to deliver radiation to the tumor based on its exact profile
• A complete surgical excision of the tumor is the treatment of choice. A partial removal of the tumor may result in heavy bleeding or tumor recurrence
• Reconstruction cosmetic surgery may be necessary after the tumor is removed
• Providing reassurance and helping with anxiety feelings

How can Juvenile Nasopharyngeal Angiofibroma be Prevented?

Currently, there are no specific methods or guidelines to prevent Juvenile Nasopharyngeal Angiofibromas.

What is the Prognosis of Juvenile Nasopharyngeal Angiofibroma? (Outcomes/Resolutions)

• Juvenile Nasopharyngeal Angiofibroma is a rare, benign (non-cancerous), but potentially serious tumor that can be locally destructive. If completely excised and removed, the prognosis is usually good
• In some cases, the tumor can recur complicating the prognosis. Further, the prognosis is affected, if the tumor invades into surrounding structures such as bone and brain tissue
• In majority of cases, a distant metastasis is not observed, unless JNA becomes malignant (seen occasionally). This can adversely impact the prognosis

Additional and Relevant Useful Information for Juvenile Nasopharyngeal Angiofibroma:

• There is no evidence to prove that oily foods and chocolate-based products have an influence on Juvenile Nasopharyngeal Angiofibroma
• JNA is not a sexually-transmitted disease. These nasal tumors are also not warts and cannot be transmitted from one individual to another