Nasopharyngeal angiofibroma

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Definitions of Nasopharyngeal angiofibroma
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List of terms related to Nasopharyngeal angiofibroma

• Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2] [3] [4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15] [16] [17] [18]

Synonyms and keywords: Juvenile nasopharyngeal angiofibroma (JNA); angiofibroma of the nasopharynx, nasopharyngeal fibroma, bleeding fibroma of adolescence, fibroangioma

Historical Perspective

• Nasopharyngeal angiofibroma was first described by Hippocrates, a Greek physician, in the 5th century BC.

• In 1940, Friedberg was the first to use the term angiofibroma.

Classification

Nasopharyngeal angiofibroma may be classified according to Fisch classification into 4 groups:

• Type I: Sphenopalatine foramen, nasopharynx, and nasal cavity are involved without bone destruction.

• Type II: Nasal sinuses or the pterygomaxillary fossa are involved with bone destruction.
• Type IIIa: Infratemporal fossa or orbit are involved without intracranial involvement
• Type IIIb: Infratemporal fossa or orbit are involved with intracranial extradural commitment.
• Type IVa: Intracranial extradural and/or intradural are involved, but optic nerve, sella, or cavernous sinus are not involved.
• Type IVb: Intracranial extradural and intradura, optic nerve, sella, and/or cavernous sinusare are involved

.

Pathophysiology

Nasopharyngeal angiofibroma is a histologically benign tumor. This tumor is located in the posterolateral wall of the nasal cavity. Nasopharyngeal angiofibroma may involve sphenoid sinuses, maxillary ethmoid, pterygoid plate , orbit, base of the skull and extradural. In rare cases, nasopharyngeal angiofibroma may involve pituitary, cavernous sinus and/or optic chiasm and anterior 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:
  • Bilobed or dumbbell-shaped with average size 3-5 cm
  • Tan to purple-red, rubbery-firm unencapsulated polypoid fibrous mass
  • Bleeding on biopsy
  • Spongy cut surface
  • Sessile or pedunculated tumor

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

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

Causes

The cause of Nasopharyngeal angiofibroma has not been identified, but it may be caused by hormonal effect. Nasopharyngeal angiofibroma express higher levels of vascular endothelial growth factor (VEGF) and hormone receptors.To review risk factors for the development of Nasopharyngeal angiofibroma, click here.

Differentiating Nasopharyngeal angiofibroma from Other Diseases

• Nasopharyngeal angiofibroma must be differentiated from other diseases that cause epistaxis, unilateral nasal obstruction, and rhinorrhea, such as:

• Sinonasal polyp
• Rhinosporidiosis
• Chordoma
• Nasopharanageal cyst
• Pyogenic granuloma
• Nasopharyngeal carcinoma
• Lymphoma,
• Rhabdomyosarcoma
• Adenoid hypertrophy

Epidemiology and Demographics

Incidence of juvenile nasopharyngeal angiofibroma is less than 0.5% of head and neck tumors.

The incidence of juvenile nasopharyngeal angiofibroma is 0.6 per 100,000 individuals worldwide.

Some reports suggest uvenile nasopharyngeal angiofibroma is more common in the indian subcontinent than in the west.

Men are more commonly affected by juvenile nasopharengeal angiofibroma than women.

Risk Factors

Common risk factors in the development of nasopharyngeal angiofibroma include:

• Familial adenomatous polyposis
• Men

Screening

There is insufficient evidence to recommend routine screening for nasopharengeal angiofibroma.

Natural History, Complications, and Prognosis

• If left untreated, % 9 of patients with nasopharengeal angiofibroma may progress to develope hemorrhage and intracranial extention.

• Common complications of nasopharengeal angiofibroma include nasal obstruction and repeated bleeding.

• Prognosis is generally good.

Diagnosis

Diagnostic Study of Choice

The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].

OR

The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].

OR

The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].

OR

There are no established criteria for the diagnosis of [disease name].

History and Symptoms

The hallmark of Nasopharyngeal angiofibroma is the classic triad of epistaxis, unilateral nasal obstruction and a mass in the nasopharynx . The most common symptoms of Nasopharyngeal angiofibroma include nasal obstruction and epistaxis and headache. Less common symptoms of Nasopharyngeal angiofibroma include conductive hearing loss, diplopia, proptosis, anosmia, recurrent ottits media and eye pain.

Physical Examination

Physical examination of patients with Nasopharyngeal angiofibroma is usually remarkable for nasal mass and rhinorrhea.

Rare physical examination include:

• Orbital mass
• Proptosis
• Zygomatic swelling
• Trismus
• Decreasing vision

Laboratory Findings

There are no diagnostic laboratory findings associated with nasopharengeal angiofibroma.

Electrocardiogram

There are no ECG findings associated with nasopharengeal angiofibroma.

X-ray

An x-ray may be helpful in the diagnosis of nasopharyngeal angiofibroma. Findings on an x-ray suggestive of include:

• Nasopharyngeal polyp
• Antral sign or Holman-Miller sign: Forward bowing of the posterior wall of the maxillary sinus
• Maxillary sinus opacification
• Bony margin may be eroded

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with nasopharengeal angiofibroma.

CT scan

CT scan demonstrate the extent of the tumor. Findings on CT scan suggestive of nasopharengeal angiofibroma include:

• Extension to the sphenoid sinus
• Erosion of the greater sphenoidal wing
• invasion of the pterygomaxillary and infratemporal fossae

MRI

Cronal MRI demonstrate the extent of the tumor especially in intracranial involvement. Findings on MRI suggestive of nasopharengeal angiofibroma include:

• Largely isointense to muscle on T1-weighted images
• Hyperintense on T2-weighted images
• Internal signal-void regions
• Intense enhancement after intravenous (IV) contrast injection

Other Imaging Findings

DSA (digital subtraction angiography) of carotid artery is helpful in the diagnosis extension of nasopharengeal angiofibroma and feeding vessel. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].extension of tumors and feeding vessels

Other Diagnostic Studies

There are no other diagnostic studies associated with [disease name].

OR

[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR   The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Surgery

Surgical intervention is not recommended for the management of [disease name].

OR

Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]

OR

The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].

OR

The feasibility of surgery depends on the stage of [malignancy] at diagnosis.

OR

Surgery is the mainstay of treatment for [disease or malignancy].

Primary Prevention

There are no established measures for the primary prevention of Nasopharyngeal angiofibroma.

Secondary Prevention

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].

References

Template:WikiDoc Sources

WikiDoc Resources for Nasopharyngeal angiofibroma
Articles
Most recent articles on Nasopharyngeal angiofibroma Most cited articles on Nasopharyngeal angiofibroma Review articles on Nasopharyngeal angiofibroma Articles on Nasopharyngeal angiofibroma in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Nasopharyngeal angiofibroma Images of Nasopharyngeal angiofibroma Photos of Nasopharyngeal angiofibroma Podcasts & MP3s on Nasopharyngeal angiofibroma Videos on Nasopharyngeal angiofibroma
Evidence Based Medicine
Cochrane Collaboration on Nasopharyngeal angiofibroma Bandolier on Nasopharyngeal angiofibroma TRIP on Nasopharyngeal angiofibroma
Clinical Trials
Ongoing Trials on Nasopharyngeal angiofibroma at Clinical Trials.gov Trial results on Nasopharyngeal angiofibroma Clinical Trials on Nasopharyngeal angiofibroma at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Nasopharyngeal angiofibroma NICE Guidance on Nasopharyngeal angiofibroma NHS PRODIGY Guidance FDA on Nasopharyngeal angiofibroma CDC on Nasopharyngeal angiofibroma
Books
Books on Nasopharyngeal angiofibroma
News
Nasopharyngeal angiofibroma in the news Be alerted to news on Nasopharyngeal angiofibroma News trends on Nasopharyngeal angiofibroma
Commentary
Blogs on Nasopharyngeal angiofibroma
Definitions
Definitions of Nasopharyngeal angiofibroma
Patient Resources / Community
Patient resources on Nasopharyngeal angiofibroma Discussion groups on Nasopharyngeal angiofibroma Patient Handouts on Nasopharyngeal angiofibroma Directions to Hospitals Treating Nasopharyngeal angiofibroma Risk calculators and risk factors for Nasopharyngeal angiofibroma
Healthcare Provider Resources
Symptoms of Nasopharyngeal angiofibroma Causes & Risk Factors for Nasopharyngeal angiofibroma Diagnostic studies for Nasopharyngeal angiofibroma Treatment of Nasopharyngeal angiofibroma
Continuing Medical Education (CME)
CME Programs on Nasopharyngeal angiofibroma
International
Nasopharyngeal angiofibroma en Espanol Nasopharyngeal angiofibroma en Francais
Business
Nasopharyngeal angiofibroma in the Marketplace Patents on Nasopharyngeal angiofibroma
Experimental / Informatics
List of terms related to Nasopharyngeal angiofibroma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [19] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [20]

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

Overview

Nasopharyngeal angiofibroma (also called juvenile nasopharyngeal angiofibroma) is a locally aggressive, benign vascular neoplasm that grows in the pterygopalatine fossa. The most common symptoms of nasopharyngeal angiofibroma include one-sided nasal obstruction and recurrent bleeding. Nasopharyngeal angiofibroma may be classified according to Radkowski Classification System (severity) into 3 categories: I, II, and III. The majority of nasopharyngeal angiofibromas are irrigated by the external carotid artery. Genetic alterations associated with the development of nasopharyngeal angiofibroma include: overexpression of PDGF-B, bFGF, VEGF. Nasopharyngeal angiofibroma accounts for 0.05% of all head and neck tumors. Nasopharyngeal angiofibromas are more commonly observed among children and adolescents. Males are more commonly affected with nasopharyngeal angiofibroma than females. Common risk factors in the development of nasopharyngeal angiofibroma, include: presence of tumor in the pterygoid fossa and young age. Physical examination may be remarkable for smooth submucosal mass in the posterior nasal cavity. Computed tomography is the imaging modality of choice. On conventional radiography, findings of nasopharyngeal angiofibroma include: visualization of a nasopharyngeal mass, opacification of the sphenoid sinus, and anterior bowing of the posterior wall of the maxillary antrum. Surgery is the mainstay of therapy for nasopharyngeal angiofibroma. Surgical approach for nasopharyngeal angiofibroma will usually depend on the stage. The treatment of choice for early stage for nasopharyngeal angiofibroma is intranasal endoscopic surgery ( e.g. lateral rhinotomy). The treatment of choice for late stage nasopharyngeal angiofibroma include the infratemporal fossa approach, and the mid-facial degloving approach. Common complications of nasopharyngeal angiofibroma include transient blindness, optic nerve damage, and low-grade consumption coagulopathy.

Historical Perspective

• Nasopharyngeal angiofibroma was first described by Hippocrates, a Greek physician, in the 5th century BC.

Classification

• Nasopharyngeal angiofibroma may be classified according to Radkowski Classification System into 3 categories:
• Stage I

• Ia: limited to nasal cavity/nasopharynx
• Ib: extension into one or more paranasal sinuses

• Stage II

• IIa: minimal extension through sphenopalatine foramen into pterygomaxillary fossa
• IIb: fills pterygomaxillary fossa bowing the posterior wall of the maxillary antrum anteriorly or extending into the orbit via the inferior orbital fissure
• IIc: extends beyond pterygomaxillary fossa into infratemporal fossa

• Stage III

• Stage IIIA: intracranial extension

Pathophysiology

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

• Vascular neoplasm
• Originates from the pterygopalatine fossa
• The majority are associated with the external carotid artery

• 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

• There are no known causes of nasopharyngeal angiofibroma.

Differentiating Nasopharyngeal Angiofibroma from Other Diseases

• Nasopharyngeal angiofibroma must be differentiated from other diseases that cause epistaxis, unilateral nasal obstruction, and rhinorrhea, such as:

• Antro-choanal polyp
• Rhinosporidiosis
• Chordoma
• Nasopharanageal cyst
• Pyogenic granuloma

Epidemiology and Demographics

• Nasopharyngeal angiofibroma is a rare tumor.
• Nasopharyngeal angiofibroma accounts for 0.05% of all head and neck tumors.
• The prevalence of nasopharyngeal angiofibroma is approximately 0.4 per 100,000 individuals worldwide.

Age

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

Gender

• The male to female ratio for nasopharyngeal angiofibroma is approximately 4 to 1.

Race

• There is no racial predilection for nasopharyngeal angiofibroma.

Risk Factors

• Common risk factors in the development of nasopharyngeal angiofibroma include:

• Presence of tumor in the pterygoid fossa
• Young age
• Feeders from the internal carotid artery
• Residual tumor

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 malignant transformation.
• Common complications of nasopharyngeal angiofibroma include transient blindness, optic nerve damage, and low-grade consumption coagulopathy.
• Prognosis is generally good, and the 5-year survival rate of patients with early stage nasopharyngeal angiofibroma is approximately 90%
• Survival rate of patients with late stage nasopharyngeal angiofibroma is approximately 50% to 75%.

Diagnosis

Diagnostic Criteria

• The diagnosis of nasopharyngeal angiofibroma is made when the following diagnostic criteria are met:

• Clinical criteria

• Young patient
• Epistaxis

• Positive physical exam

• A smooth submucosal mass in the posterior nasal cavity

• Positive imaging finding: visualization of a nasopharyngeal mass

Symptoms

• Common symptoms of nasopharyngeal angiofibroma, may include:

• Epistaxis or blood-tinged nasal discharge
• Unilateral nasal obstruction
• Rhinorrhea
• Hearing loss
• Diplopia
• Anosmia (rare)
• 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

• There are no specific laboratory findings associated with the diagnosis of nasopharyngeal angiofibroma.

Imaging Findings

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

• Visualization 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
• Anterior bowing the posterior wall of the maxillary antrum

• 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 nasal endoscopy.

Treatment

Medical Therapy

• Medical therapy for nasopharyngeal angiofibroma is divided into 2 categories:

• Hormonal therapy
• Radiotherapy

• Hormonal therapy for nasopharyngeal angiofibroma includes:

• Flutamide

• Medical treatment is usually given before surgery to reduce the blood loss
• Radiotherapy for nasopharyngeal angiofibroma, include:

• Stereotactic radiotherapy

Surgery

• Surgery is the mainstay of therapy for nasopharyngeal angiofibroma.
• Preoperative embolization is highly suggested for nasopharyngeal angiofibroma.
• Surgical approach for nasopharyngeal angiofibroma will depend on the stage.

• The treatment of choice for early stage for nasopharyngeal angiofibroma is intranasal endoscopic surgery. Lateral rhinotomy is the preferred surgical approach.
• The treatment of choice for late stage for nasopharyngeal angiofibroma include the infratemporal fossa approach, and the mid-facial degloving approach.

Prevention

• There are no primary preventive measures available for nasopharyngeal angiofibroma.
• Once diagnosed and successfully treated, patients with nasopharyngeal angiofibroma are followed-up after surgery, and every 6 or 12 months, depending on the clinical progression.