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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Juvenile nasopharyngeal angiofibroma; angiofibroma of the nasopharynx;

Overview

Nasopharyngeal angiofibroma (also called juvenile nasopharyngeal angiofibroma) is a histologically benign but locally aggressive vascular tumor that grows in the back of the nasal cavity. It almost exclusively affects adolescent males. Patients with nasopharyngeal angiofibroma usually present with one-sided nasal obstruction and recurrent bleeding.

Historical Perspective

• Nasopharyngeal angiofibroma was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].

Classification

• Nasopharyngeal angiofibroma may be classified according to [classification method] into [number] subtypes/groups:

• [group1]
• [group2]
• [group3]

• Other variants of nasopharyngeal angiofibroma include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

• The pathogenesis of nasopharyngeal angiofibroma is characterized by the following features:

• Vascular neoplasm
• Originates from the pterygopalatine fossa

• Genetic alterations associated with the development of nasopharyngeal angiofibroma, include:

• Overexpression PDGF-B
• Overexpression bFGF
• Overexpression bFGF
• Deletion of chromosome 17
• Tumor suppressor gene p53
• Overexpression of Her-2/neu oncogene

• On gross pathology, characteristic findings of nasopharyngeal angiofibroma, include:

• Unencapsulated
• Polypoid fibrous mass
• Bleeding on manipulation

• On microscopic histopathological analysis, characteristic findings of nasopharyngeal angiofibroma, include:

• Fibroblastic cells with plump (near cuboidal) nuclei
• Fibrous stroma
• Abundant capillaries

Causes

• Common causes of nasopharyngeal angiofibroma, include:

Differentiating Nasopharyngeal Angiofibroma from Other Diseases

• Nasopharyngeal angiofibroma must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:

• Antro-choanal polyp (antral-choanal polyp)
• Rhinosporidiosis
• Malignancy
• Chordoma
• Nasopharanageal cyst
• Pyogenic granuloma

Epidemiology and Demographics

• Nasopharyngeal angiofibroma is rare
• Nasopharyngeal angiofibroma accounts for 0.05% of all head and neck tumors.
• The prevalence of nasopharyngeal angiofibroma is approximately 1 per 100,000 individuals worldwide.

Age

• Nasopharyngeal angiofibroma is more commonly observed among patients aged 7-19 years
• Nasopharyngeal angiofibroma is more commonly observed among children and adolescents .

Gender

• Males are more commonly affected with nasopharyngeal angiofibroma than females.
• The male to female ratio for nasopharyngeal angiofibroma is approximately 4 to 1.

Race

• There is no racial predilection for nasopharyngeal angiofibroma.

• Nasopharyngeal angiofibroma usually affects individuals of the [race 1] race.
• [Race 2] individuals are less likely to develop nasopharyngeal angiofibroma.

Risk Factors

• Common risk factors in the development of nasopharyngeal angiofibroma are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

• The majority of patients with nasopharyngeal angiofibroma are symptomatic at diagnosis.
• Early clinical features include epistaxis, facial pain, and headache.
• If left untreated, the majority of patients with nasopharyngeal angiofibroma may progress to develop

• The most common complication of nasopharyngeal angiofibroma is

• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with nasopharyngeal angiofibroma is approximately [#%].

Diagnosis

Diagnostic Criteria

• The diagnosis of nasopharyngeal angiofibroma is made when at least [number] of the following [number] diagnostic criteria are met:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

Symptoms

• Common symptoms of nasopharyngeal angiofibroma, may include:

• Epistaxis or blood-tinged nasal discharge
• Unilateral nasal obstruction
• Rhinorrhea
• Hearing loss
• Diplopia
• Rarely anosmia
• Eye pain

Physical Examination

• Patients with nasopharyngeal angiofibroma usually are well-appearing.
• Physical examination may be remarkable for:

• A smooth submucosal mass in the posterior nasal cavity

Laboratory Findings

• Laboratory findings consistent with the diagnosis of nasopharyngeal angiofibroma, include:

Imaging Findings

• Computed tomography is the imaging modality of choice for nasopharyngeal angiofibroma.
• On conventional radiography, findings of nasopharyngeal angiofibroma, include:

• Visualisation of a nasopharyngeal mass
• Opacification of the sphenoid sinus
• Anterior bowing of the posterior wall of the maxillary antrum
• Holman-miller sign: the anterior bowing of the posterior wall of the maxillary antrum which is seen on lateral skull film or cross-sectional imaging
• Widening of the pterygomaxillary fissure and pterygopalatine fossa
• Erosion of the medial pterygoid plate

• On CT, findings of nasopharyngeal angiofibroma, include:

• Bony changes
• Non-encapsulated soft tissue mass
• Bowing the posterior wall of the maxillary antrum anteriorly

• On MRI, findings of nasopharyngeal angiofibroma, include:

• T1: intermediate signal
• T2: heterogeneous signal: flow voids appear dark
• T1 C+ (Gd): shows prominent enhancement

• The images below demonstrate findings of nasopharyngeal angiofibroma.

• 

  Nasopharyngeal angiofibroma. Skull base invasionImage courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

• 

  MRI (T1): Nasopharyngeal angiofibroma 1/3 Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

• 

  MRI (T1): Nasopharyngeal angiofibroma Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

• 

  MRI (T1): Nasopharyngeal angiofibroma 3/3 Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

• 

  MRI (T1): Nasopharyngeal angiofibroma 3/3 (circled)Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

Other Diagnostic Studies

• Nasopharyngeal angiofibroma may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• There is no treatment for nasopharyngeal angiofibroma; the mainstay of therapy is supportive care.

• The mainstay of therapy for nasopharyngeal angiofibroma is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for nasopharyngeal angiofibroma.
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of nasopharyngeal angiofibroma.
• [Surgical procedure] can only be performed for patients with [disease stage] nasopharyngeal angiofibroma.

Prevention

• There are no primary preventive measures available for nasopharyngeal angiofibroma.

• Effective measures for the primary prevention of nasopharyngeal angiofibroma include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with nasopharyngeal angiofibroma are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].