Juvenile nasopharyngeal angiofibroma (JNA) is a rare vascular tumor: a condition in which there is an overgrowth of vessels. This tumor or growth grows in the nose and paranasal sinuses. This tumor is mainly seen in young people, hence the name ‘juvenile’ (which means ‘youth’). A juvenile nasopharyngeal angiofibroma can range from an easily bleeding and relatively small tumor inside the nose, which may be visible in the nostrils, to a destructive tumor in the paranasal sinuses that is difficult to remove surgically. Treatment of juvenile nasopharyngeal angiofibroma consists of removing the entire tumor.
Juvenile nasopharyngeal angiofibroma
- What is juvenile nasopharyngeal angiofibroma?
- Who gets juvenile nasopharyngeal angiofibroma?
- What is the cause of this tumor or growth in the nose?
- What are the symptoms of JNA?
- How is JNA diagnosed?
- Diagnostic research
- Differential diagnosis
- How is JNA treated?
- Tissue research
What is juvenile nasopharyngeal angiofibroma?
Juvenile nasopharyngeal angiofibroma (JNA) is a benign tumor of the nasal cavity that commonly occurs in teenage boys. It is an angiofibroma, a benign tumor made up of vascular and connective tissue. Although this tumor is not malignant (cancerous), it can grow into a tumor of significantly large size and spread into the paranasal sinuses and various compartments in the head making it a difficult tumor to treat.
Who gets juvenile nasopharyngeal angiofibroma?
Juvenile nasopharyngeal angiofibroma is a rare condition. The tumor comprises only 0.05% of all head and neck tumors. JNA is almost exclusively seen in men/boys aged 7-19 years. The tumor rarely occurs in women. People of all racial and ethnic backgrounds can develop this tumor; no geographical preference was observed.
What is the cause of this tumor or growth in the nose?
The cause of juvenile nasopharyngeal angiofibroma is related to hormonal influences, such as androgenic hormones, the collective name for all male hormones. Since significant hormonal fluctuations occur during puberty in boys, this supports the theory of hormonal influence as a causal factor for this tumor growth and development. The most important endogenous androgenic hormone is testosterone, produced mainly in the testes and to a lesser extent in the adrenal glands. In women, the ovaries and adrenal glands produce small amounts of this hormone. This substance is necessary for the libido (sex drive), the ovulation process (ovulation or ovulation is the release of an egg from the ovary) and body hair.
What are the symptoms of JNA?
The symptoms usually include nasal congestion (a stuffy nose), nosebleeds and sometimes difficulty breathing (through the nose). Occasionally headaches and facial swelling may occur. A nosebleed from only one nostril is common for tumors such as JNA and should always be examined by a doctor.The tumor often has a size between 0.5 to 5 cm, usually in the nose (nasal cavity or nasopharyngeal region). Sometimes the tumor is visible in the nostrils. However, some tumors are aggressive and invade surrounding structures, affecting their function. In that case, you may experience additional complaints such as:
- decreased sense of smell or loss of sense of smell;
- deafness, earache; and
- facial swelling or deformity.
CT scan / Source: IStock.com/Pavel Losevsky
How is JNA diagnosed?
The following diagnostic methods can be used to diagnose juvenile nasopharyngeal angiofibroma:
- A thorough physical examination and review of a person’s complete medical history are very crucial;
- Radiological examinations of the head and neck area that include X-rays, CT or MRI scans;
- Vascular angiography: an X-ray examination in which contrast fluid is injected directly into the blood vessels, making the course of the veins visible and any abnormalities .
Nasal polyps / Source: MathieuMD , Wikimedia Commons (CC BY-SA-3.0)
Many clinical conditions can have similar symptoms:
- Other causes of nasal obstruction, for example, nasal polyps, antrochoanal polyp, teratoma, encephalocele (a congenital disorder of the skull), squamous cell carcinoma (a form of skin cancer), etc.;
- Other causes of nosebleed (epistaxis), systemic or local;
- Other causes of facial swelling.
How is JNA treated?
Surgery is the preferred treatment method. In the past, surgical treatment sometimes involved major incisions in the face or mouth, but more recently techniques are being used that require less invasive incisions and allow the tumor to be approached through the nose with advanced endoscopic sinus surgery techniques. The operation is performed under general anesthesia. The surgeon performing this operation must have significant experience in endoscopic sinus surgery.
The excised tumor is sent to a laboratory for pathological examination. The pathologist examines the tumor under a microscope. The definitive diagnosis is then also determined. The tumor should not be partially biopsied because it may bleed heavily during excision. Therefore, it must be completely removed at once as part of the biopsy.
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