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{{CMG}} {{AE}} {{MV}}
{{CMG}} {{AE}} {{MV}}
   
   
{{SK}} Juvenile nasopharyngeal angiofibroma; angiofibroma of the nasopharynx;  
{{SK}} Juvenile nasopharyngeal angiofibroma; angiofibroma of the nasopharynx; JNA
   
   
==Overview==
==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.
'''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. Nasopharyngeal angiofibroma may be classified according to Radkowski Classification System into 3 categories: I, II, and III.  Nasopharyngeal angiofibroma is a vascular neoplasm, originates from the pterygopalatine fossa.  The majority of nasopharyngeal angiofibromas are irrigated by the external carotid artery.  Nasopharyngeal angiofibroma is rare, it account for 0.05% of all head and neck tumors. Nasopharyngeal angiofibromas are more commonly observed among  children and adolescents. Common risk factors in the development of nasopharyngeal angiofibroma, include:
presence of tumor in the pterygoid fossa and young age. Early clinical features include epistaxis, facial pain, and headache. The most common symptom is unilateral nasal obstruction.  


==Historical Perspective==
==Historical Perspective==
*Nasopharyngeal angiofibroma was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
*Nasopharyngeal angiofibroma was first described by Hippocrates, a Greek physician, in the 5th century BC.


==Classification==
==Classification==
*Nasopharyngeal angiofibroma may be classified according to [classification method] into [number] subtypes/groups:
*Nasopharyngeal angiofibroma may be classified according to Radkowski Classification System into 3 categories:
:*[group1]
*'''Stage I'''
:*[group2]
:*Ia: limited to nasal cavity/nasopharynx
:*[group3]
:*Ib: extension into one or more paranasal sinuses
*Other variants of nasopharyngeal angiofibroma include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
*'''Stage II'''
:*IIa: minimal extension through sphenopalatine foramen into pterygomaxillary fossa
:*IIb: fills pterygomaxillary fossa bowing the posterior wall of the maxiallary 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==
==Pathophysiology==
*The pathogenesis of nasopharyngeal angiofibroma is characterized by the following features:  
*The pathogenesis of nasopharyngeal angiofibroma is characterized by the following features:<ref name="pmid11374252">{{cite journal |vauthors=Paris J, Guelfucci B, Moulin G, Zanaret M, Triglia JM |title=Diagnosis and treatment of juvenile nasopharyngeal angiofibroma |journal=Eur Arch Otorhinolaryngol |volume=258 |issue=3 |pages=120–4 |year=2001 |pmid=11374252 |doi= |url=}}</ref>
:*Vascular neoplasm  
:*Vascular neoplasm  
:*Originates from the pterygopalatine fossa  
:*Originates from the pterygopalatine fossa  
:*The majority are associated with the external carotid artery
*Genetic alterations  associated with the development of nasopharyngeal angiofibroma, include:<ref name="pmid18228521">{{cite journal |vauthors=Coutinho-Camillo CM, Brentani MM, Nagai MA |title=Genetic alterations in juvenile nasopharyngeal angiofibromas |journal=Head Neck |volume=30 |issue=3 |pages=390–400 |year=2008 |pmid=18228521 |doi=10.1002/hed.20775 |url=}}</ref>
*Genetic alterations  associated with the development of nasopharyngeal angiofibroma, include:<ref name="pmid18228521">{{cite journal |vauthors=Coutinho-Camillo CM, Brentani MM, Nagai MA |title=Genetic alterations in juvenile nasopharyngeal angiofibromas |journal=Head Neck |volume=30 |issue=3 |pages=390–400 |year=2008 |pmid=18228521 |doi=10.1002/hed.20775 |url=}}</ref>
:*Overexpression PDGF-B  
:*Overexpression PDGF-B  
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==Causes==
==Causes==
* Common causes of nasopharyngeal angiofibroma, include:
* There are no known causes of nasopharyngeal angiofibroma.


==Differentiating Nasopharyngeal Angiofibroma from Other Diseases==
==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:
*Nasopharyngeal angiofibroma must be differentiated from other diseases that cause epistaxis, unilateral nasal obstruction, and rhinorrhea, such as:
:* Antro-choanal polyp (antral-choanal polyp)
:* Antro-choanal polyp (antral-choanal polyp)
:* Rhinosporidiosis  
:* Rhinosporidiosis  
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:* Nasopharanageal cyst
:* Nasopharanageal cyst
:* Pyogenic granuloma
:* Pyogenic granuloma
 
==Epidemiology and Demographics==
==Epidemiology and Demographics==
:* Nasopharyngeal angiofibroma is rare
* Nasopharyngeal angiofibroma is rare
:* Nasopharyngeal angiofibroma accounts for 0.05% of all head and neck tumors.
* 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.
* The prevalence of nasopharyngeal angiofibroma is approximately 1 per 100,000 individuals worldwide.


===Age===
===Age===
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===Gender===
===Gender===
*Males are more commonly affected with nasopharyngeal angiofibroma than females.
*Males are more commonly affected with nasopharyngeal angiofibroma than females.
*The male to female ratio for nasopharyngeal angiofibroma is approximately 4 to 1.
*The male to female ratio for nasopharyngeal angiofibroma is approximately 4 to 1.
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===Race===
===Race===
*There is no racial predilection for nasopharyngeal angiofibroma.
*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==
==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].
*Common risk factors in the development of nasopharyngeal angiofibroma, include:<ref name="pmid11374252">{{cite journal |vauthors=Paris J, Guelfucci B, Moulin G, Zanaret M, Triglia JM |title=Diagnosis and treatment of juvenile nasopharyngeal angiofibroma |journal=Eur Arch Otorhinolaryngol |volume=258 |issue=3 |pages=120–4 |year=2001 |pmid=11374252 |doi= |url=}}</ref>
:*Presence of tumor in the pterygoid fossa
:*Young age
:*Feeders from the internal carotid artery
:*Residual tumor
 
== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*The majority of patients with nasopharyngeal angiofibroma are symptomatic at diagnosis.  
*The majority of patients with nasopharyngeal angiofibroma are symptomatic at diagnosis.  
*Early clinical features include epistaxis, facial pain, and headache.
*Early clinical features include epistaxis, facial pain, and headache.
*If left untreated, the majority of patients with nasopharyngeal angiofibroma may progress to develop
*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 40%


*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 ==
== Diagnosis ==
===Diagnostic Criteria===
===Diagnostic Criteria===
*The diagnosis of nasopharyngeal angiofibroma is made when at least [number] of the following [number] diagnostic criteria are met:
*The diagnosis of nasopharyngeal angiofibroma is with the following diagnostic criteria:<ref name="pmid11374252">{{cite journal |vauthors=Paris J, Guelfucci B, Moulin G, Zanaret M, Triglia JM |title=Diagnosis and treatment of juvenile nasopharyngeal angiofibroma |journal=Eur Arch Otorhinolaryngol |volume=258 |issue=3 |pages=120–4 |year=2001 |pmid=11374252 |doi= |url=}}</ref>
:*[criterion 1]
:*Clinical criteria
:*[criterion 2]
::*Young patient
:*[criterion 3]
::*Epistaxis
:*[criterion 4]
:*Positive physical exam
::*A smooth submucosal mass in the posterior nasal cavity
:*Positive imaging finding: visualisation of a nasopharyngeal mass
 
=== Symptoms ===
=== Symptoms ===
*Common symptoms of nasopharyngeal angiofibroma, may include:
*Common symptoms of nasopharyngeal angiofibroma, may include:<ref name="pmid11374252">{{cite journal |vauthors=Paris J, Guelfucci B, Moulin G, Zanaret M, Triglia JM |title=Diagnosis and treatment of juvenile nasopharyngeal angiofibroma |journal=Eur Arch Otorhinolaryngol |volume=258 |issue=3 |pages=120–4 |year=2001 |pmid=11374252 |doi= |url=}}</ref>
:*Epistaxis or blood-tinged nasal discharge
:*Epistaxis or blood-tinged nasal discharge
:*Unilateral nasal obstruction  
:*Unilateral nasal obstruction  
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=== Laboratory Findings ===
=== Laboratory Findings ===
*Laboratory findings consistent with the diagnosis of nasopharyngeal angiofibroma, include:
*There are no specific laboratory findings associated with the diagnosis of nasopharyngeal angiofibroma.
 
 
 
   
   
===Imaging Findings===
===Imaging Findings===
*Computed tomography is the imaging modality of choice for nasopharyngeal angiofibroma.
*Computed tomography is the imaging modality of choice for nasopharyngeal angiofibroma.<ref name="pmid11374252">{{cite journal |vauthors=Paris J, Guelfucci B, Moulin G, Zanaret M, Triglia JM |title=Diagnosis and treatment of juvenile nasopharyngeal angiofibroma |journal=Eur Arch Otorhinolaryngol |volume=258 |issue=3 |pages=120–4 |year=2001 |pmid=11374252 |doi= |url=}}</ref>
*On conventional radiography, findings of nasopharyngeal angiofibroma, include:  
*On conventional radiography, findings of nasopharyngeal angiofibroma, include:  
:*Visualisation of a nasopharyngeal mass
:*Visualisation of a nasopharyngeal mass
Line 140: Line 144:


=== Other Diagnostic Studies ===
=== Other Diagnostic Studies ===
*Nasopharyngeal angiofibroma may also be diagnosed using [diagnostic study name].
*Nasopharyngeal angiofibroma may also be diagnosed using nasal [[endoscopy]].  
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
   
   
== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*There is no treatment for nasopharyngeal angiofibroma; the mainstay of therapy is supportive care.
*Medical therapy for nasopharyngeal angiofibroma is divided into 2 categories:<ref name="pmid11374252">{{cite journal |vauthors=Paris J, Guelfucci B, Moulin G, Zanaret M, Triglia JM |title=Diagnosis and treatment of juvenile nasopharyngeal angiofibroma |journal=Eur Arch Otorhinolaryngol |volume=258 |issue=3 |pages=120–4 |year=2001 |pmid=11374252 |doi= |url=}}</ref>
:*Hormonal therapy
*The mainstay of therapy for nasopharyngeal angiofibroma is [medical therapy 1] and [medical therapy 2].
:*Radiotherapy
*[Medical therapy 1] acts by [mechanism of action1].
*Hormonal therapy for nasopharyngeal angiofibroma, includes:
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
:*Flutamide
*Medical treatment is usually given before surgery to reduce the blood loss
*Radiotherapy for nasopharyngeal angiofibroma, include:
:*Stereotactic radiotherapy
 
=== Surgery ===
=== Surgery ===
*Surgery is the mainstay of therapy for nasopharyngeal angiofibroma.
*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 approach for nasopharyngeal angiofibroma will depend on the stage.
*[Surgical procedure] can only be performed for patients with [disease stage] nasopharyngeal angiofibroma.
:*The treatment of choice  for early stage for nasopharyngeal angiofibroma is intranasal endoscopic surgery. Lateral rhinotomy is the usual preferred surgical approach of choice.  
:*The treatment of choice  for late stage for nasopharyngeal angiofibroma include the infratemporal fossa approach, and the mid-facial degloving approach.  
 
=== Prevention ===
=== Prevention ===
*There are no primary preventive measures available for nasopharyngeal angiofibroma.
*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 3, 6, or 12 months.  
*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].  


==References==
==References==

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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; JNA

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. Nasopharyngeal angiofibroma may be classified according to Radkowski Classification System into 3 categories: I, II, and III. Nasopharyngeal angiofibroma is a vascular neoplasm, originates from the pterygopalatine fossa. The majority of nasopharyngeal angiofibromas are irrigated by the external carotid artery. Nasopharyngeal angiofibroma is rare, it account for 0.05% of all head and neck tumors. Nasopharyngeal angiofibromas are more commonly observed among children and adolescents. Common risk factors in the development of nasopharyngeal angiofibroma, include: presence of tumor in the pterygoid fossa and young age. Early clinical features include epistaxis, facial pain, and headache. The most common symptom is unilateral nasal obstruction.

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 maxiallary 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:[1]
  • 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:[2]
  • 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 (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.

Risk Factors

  • Common risk factors in the development of nasopharyngeal angiofibroma, include:[1]
  • 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 40%

Diagnosis

Diagnostic Criteria

  • The diagnosis of nasopharyngeal angiofibroma is with the following diagnostic criteria:[1]
  • Clinical criteria
  • Young patient
  • Epistaxis
  • Positive physical exam
  • A smooth submucosal mass in the posterior nasal cavity
  • Positive imaging finding: visualisation of a nasopharyngeal mass

Symptoms

  • Common symptoms of nasopharyngeal angiofibroma, may include:[1]
  • 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

  • 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.[1]
  • 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.

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:[1]
  • 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.
  • 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 usual preferred surgical approach of choice.
  • 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 3, 6, or 12 months.

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 1.5 Paris J, Guelfucci B, Moulin G, Zanaret M, Triglia JM (2001). "Diagnosis and treatment of juvenile nasopharyngeal angiofibroma". Eur Arch Otorhinolaryngol. 258 (3): 120–4. PMID 11374252.
  2. ↑ Coutinho-Camillo CM, Brentani MM, Nagai MA (2008). "Genetic alterations in juvenile nasopharyngeal angiofibromas". Head Neck. 30 (3): 390–400. doi:10.1002/hed.20775. PMID 18228521.
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