Juvenile nasopharyngeal angiofibroma

What it is

From: Orphanet

Juvenile nasopharyngeal angiofibroma (JNA) is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the nasopharynx, which affects mainly young and adolescent males (onset usually occurring between 7-19 years of age) and that presents as a mass in the nasopharynx and nasal cavity, leading to manifestations such as nasal obstruction, epistaxis, profound facial swelling, proptosis or diplopia. Although slowly progressive, it has a high rate of recurrence and sometimes invades adjacent structures.

Reported clinical features and what the terms mean

The following findings are associated with this condition in Orphanet. They are not a checklist for diagnosing yourself, and they do not all occur in every affected person. Some are examination, imaging or laboratory findings that cannot be recognised at home.

The frequency labels describe how often a finding was reported among people with the condition in the source. They do not give the chance that a person with that symptom has the condition. Definitions below reproduce HPO terminology; they explain the term, not the likely severity in an individual.

Juvenile nasopharyngeal angiofibroma · Obligate (100%)

A benign but highly vascular nasopharyngeal neoplasm. The tumor originates from the sphenopalatine foramen and involves both the pterygopalatine fossa and the posterior nasal cavity.

Diplopia · Frequent (79-30%)

Diplopia is a condition in which a single object is perceived as two images, it is also known as double vision.

Epistaxis · Frequent (79-30%)

Epistaxis, or nosebleed, refers to a hemorrhage localized in the nose.

Nasal congestion · Frequent (79-30%)

Reduced ability to pass air through the nasal cavity often leading to mouth breathing.

Proptosis · Frequent (79-30%)

An eye that is protruding anterior to the plane of the face to a greater extent than is typical.

Facial edema · Frequent (79-30%)

Increased intracranial pressure · Occasional (29-5%)

An increase of the pressure inside the cranium (skull) and thereby in the brain tissue and cerebrospinal fluid.

Juvenile colonic polyposis · Very rare (<4-1%)

The presence of more than 5 juvenile polyps of the colon. The term juvenile polyps refer to a special histopathology and not the age of onset as the polyp might be diagnosed at all ages. The juvenile polyp has a spherical appearance and is microscopically characterized by overgrowth of an oedematous lamina propria with inflammatory cells and cystic glands.

When it may begin

From: Orphanet

Adolescent; Childhood

Inheritance in the source

From: Orphanet

Not applicable

Frequency and the population described

From: Orphanet

Point prevalence: Unknown; Worldwide; Class only. Annual incidence: 1-9 / 1 000 000; Worldwide; Value and class.

Which doctor should you see?

The suggested department for discussing Juvenile nasopharyngeal angiofibroma is ENT, with a ent specialist / otorhinolaryngologist as the relevant type of clinician. ENT specialist / Otorhinolaryngologist; paediatric services for children as appropriate.

This is an editorial referral starting point. The appropriate clinic depends on the person’s age, symptoms, previous diagnosis and local services. The first clinician can decide whether another specialty or a team is needed; a department label does not confirm the diagnosis.

How to prepare for an assessment

Bring a short timeline of the main symptoms: when they first appeared, whether they are constant or episodic, what seems to change them, and how they affect daily activities. Include previous reports, discharge summaries, current medicines and supplements, allergies, and any relevant family history. A dated record is more useful than trying to match every feature in an online article.

Ask the clinician what is already established and what remains uncertain. If a test is suggested, ask what question it answers, what its limitations are and how the result would change the next step. The information here is not an instruction to arrange every possible test. In children, bring growth, developmental and school information if it is relevant to the concern.

• Is the main issue hearing, balance, the nose, the throat or another structure?
• Which changes in swallowing, voice or breathing need prompt attention?
• Would hearing, speech or other rehabilitation support be useful?

Treatment discussions and follow-up

The material gathered for this draft does not provide a complete condition-specific treatment pathway for Juvenile nasopharyngeal angiofibroma. That gap does not mean that treatment is unavailable. A clinician needs to establish the diagnosis and review current guidance before recommending medicines, procedures, rehabilitation or other support.

Before leaving the appointment, clarify the next review date, who will communicate results, and whom to contact if the situation changes. Discuss difficulties with sleep, work, school, mobility, eating or emotional wellbeing when these are relevant. Practical support may require coordination between the treating clinician and other services.

The collected references do not establish a complete prevention or long-term outlook section for this entry. Missing information should not be interpreted as proof that prevention is impossible or that a particular outcome is inevitable. Ask what is known for the exact subtype, stage and personal circumstances, and which uncertainties remain.

When to seek emergency help

Severe breathing difficulty, collapse, new stroke-like symptoms, a seizure that is prolonged or repeated without recovery, uncontrolled major bleeding, or an immediate risk of self-harm require emergency help. In India, call 112 or reach the nearest emergency department. This is a general, non-exhaustive warning list; it is not a condition-specific triage tool.