Nasopharyngeal angiofibroma: Difference between revisions

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{{SK}} Juvenile nasopharyngeal angiofibroma; angiofibroma of the nasopharynx; JNA
{{SK}} Juvenile nasopharyngeal angiofibroma (JNA); [[angiofibroma]] of the [[nasopharynx]], nasopharyngeal fibroma, bleeding [[fibroma]] of adolescence, fibroangioma
 
==Overview==
==Overview==
 
Nasopharyngeal angiofibroma is a benign, vascular, polypoid [[fibrous]] mass in located in the the posterolateral wall of the [[nasal cavity]]. It was first described by Hippocrates, a Greek physician, in the 5th century BC. Nasopharyngeal angiofibroma may be classified according to Fisch classification into 4 groups. Nasopharyngeal angiofibroma is a histologically [[benign]] [[tumor]]. This tumor is located in the posterolateral wall of the [[nasal cavity]]. On gross pathology, characteristic findings of nasopharyngeal angiofibroma include tan to purple-red, rubbery-firm unencapsulated polypoid [[fibrous]] mass. [[Fibroblastic]] cells with plump (near cuboidal) [[nuclei]] and abundant [[capillaries]] are seen on microscopic histopathological analysis. The cause of Nasopharyngeal angiofibroma has not been identified, but it may be caused by [[hormonal]] effect. [[Incidence]] of juvenile nasopharyngeal angiofibroma is less than 0.5% of head and neck [[Tumor|tumors]]. If left untreated, %9 of patients with nasopharyngeal angiofibroma may progress to develop [[hemorrhage]] and intracranial extension. The hallmark of Nasopharyngeal angiofibroma is the classic triad of [[epistaxis]], unilateral nasal obstruction and a mass in the [[nasopharynx]]. Physical examination of patients with Nasopharyngeal angiofibroma is usually remarkable for nasal mass and [[rhinorrhea]]. Findings on an x-ray suggestive of nasopharyngeal angiofibroma include nasopharyngeal [[polyp]] and Antral sign or Holman-Miller sign (Forward bowing of the [[posterior]] wall of the [[maxillary sinus]]). CT scan and MRI demonstrate the extent of the tumor. The mainstay of treatment for nasopharyngeal angiofibroma is [[surgical resection]].
'''Nasopharyngeal angiofibroma''' (also called juvenile nasopharyngeal angiofibroma) is a locally aggressive, benign vascular neoplasm that grows in the the pterygopalatine fossa.  The most common symptoms of nasopharyngeal angiofibroma include one-sided nasal obstruction and [[Bleeding|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, [[Vascular endothelial growth factor|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 for nasopharyngeal angiofibroma. On conventional radiography, findings of nasopharyngeal angiofibroma, include: visualisation 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.( eg. lateral rhinotomy). The treatment of choice  for late stage for nasopharyngeal angiofibroma include the infratemporal fossa approach, and the mid-facial degloving approach. Common complications of nasopharyngeal angiofibroma include [[Blindness|transient blindness]], optic nerve damage, and [[Consumption coagulopathy|low-grade consumption coagulopathy]].  


==Historical Perspective==
==Historical Perspective==
*Nasopharyngeal angiofibroma was first described by Hippocrates, a Greek physician, in the 5th century BC.
* Nasopharyngeal angiofibroma was first described by Hippocrates, a Greek physician, in the 5th century BC.<ref name="pmid22319699">{{cite journal |vauthors=Moorthy PN, Ranganatha Reddy B, Qaiyum HA, Madhira S, Kolloju S |title=Management of juvenile nasopharyngeal angiofibroma: a five year retrospective study |journal=Indian J Otolaryngol Head Neck Surg |volume=62 |issue=4 |pages=390–4 |date=October 2010 |pmid=22319699 |pmc=3266082 |doi=10.1007/s12070-010-0097-2 |url=}}</ref>


* In 1940, Friedberg was the first to use the term angiofibroma.
* The first [[endoscopic]] [[resection]] of nasopharyngeal angiofibroma was done in 1996. 
==Classification==
==Classification==
*Nasopharyngeal angiofibroma may be classified according to Radkowski Classification System into 3 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>
Nasopharyngeal angiofibroma may be classified according to '''Fisch classification''' into 4 groups: <ref name="pmid25991988">{{cite journal |vauthors=Martins MB, de Lima FV, Mendonça CA, de Jesus EP, Santos AC, Barreto VM, Santos RC |title=Nasopharyngeal angiofibroma: Our experience and literature review |journal=Int Arch Otorhinolaryngol |volume=17 |issue=1 |pages=14–9 |date=January 2013 |pmid=25991988 |pmc=4423317 |doi=10.7162/S1809-97772013000100003 |url=}}</ref>
*'''Stage I'''
* '''Type I''': [[Sphenopalatine foramen]], [[nasopharynx]], and nasal cavity are involved without [[bone]] destruction.
:*Ia: limited to nasal cavity/nasopharynx
 
:*Ib: extension into one or more paranasal sinuses
* '''Type II''': Nasal [[sinuses]] or the pterygomaxillary fossa are involved with [[bone]] destruction.
*'''Stage II'''
* '''Type IIIa''': [[Infratemporal fossa]] or [[orbit]] are involved without intracranial involvement.
:*IIa: minimal extension through [[sphenopalatine foramen]] into pterygomaxillary fossa
* '''Type IIIb''': Infratemporal fossa or [[orbit]] are involved with intracranial extradural commitment.
:*IIb: fills pterygomaxillary fossa bowing the posterior wall of the maxillary antrum anteriorly or extending into the orbit via the inferior orbital fissure.
* '''Type IVa''': Intracranial extradural and/or intradural are involved, but [[optic nerve]], [[sella]], or [[cavernous sinus]] are not involved.
:*IIc: extends beyond pterygomaxillary fossa into infratemporal fossa  
* '''Type IVb''': Intracranial extradural and intradura, [[optic nerve]], [[sella]], and/or [[cavernous sinus]] are involved.
*'''Stage III'''
:*Stage IIIA: intracranial extension


==Pathophysiology==
==Pathophysiology==
*The pathogenesis of nasopharyngeal angiofibroma is characterized by the following features:
* Nasopharyngeal angiofibroma is a histologically [[benign]] [[tumor]].
:*Vascular neoplasm
 
:*Originates from the pterygopalatine fossa
* This tumor is located in the posterolateral wall of the [[nasal cavity]].
:*The majority are associated with the [[external carotid artery]]
* Nasopharyngeal angiofibroma may involve [[sphenoid]] sinuses, [[maxillary]], [[ethmoid]], [[pterygoid plate]], [[orbit]], [[base of the skull]] and extradural.
*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>
* In rare cases, nasopharyngeal angiofibroma may involve [[pituitary]], [[cavernous sinus]] and/or [[optic chiasm]] and anterior [[fossa]]. <ref name="pmid15995507">{{cite journal |vauthors=Pryor SG, Moore EJ, Kasperbauer JL |title=Endoscopic versus traditional approaches for excision of juvenile nasopharyngeal angiofibroma |journal=Laryngoscope |volume=115 |issue=7 |pages=1201–7 |date=July 2005 |pmid=15995507 |doi=10.1097/01.MLG.0000162655.96247.66 |url=}}</ref>
 
*Genetic alterations associated with the development of nasopharyngeal angiofibroma include:<ref name="pmid18228521" />
:*Overexpression PDGF-B  
:*Overexpression PDGF-B  
:*Overexpression bFGF
:*Overexpression [[Basic fibroblast growth factor|BFGF]]
:*Overexpression bFGF
:*Deletion of chromosome 17
:*Deletion of chromosome 17
:*Tumor suppressor gene p53  
:*[[Tumor suppressor gene]] p53  
:*Overexpression of Her-2/neu oncogene
:*Overexpression of Her-2/neu [[oncogene]]
*On gross pathology, characteristic findings of nasopharyngeal angiofibroma, include:  
*On gross pathology, characteristic findings of nasopharyngeal angiofibroma include:
:*Unencapsulated
**Bilobed or dumbbell-shaped with average size 3-5 cm  <ref name="pmid28632604">{{cite journal |vauthors=McKnight CD, Parmar HA, Watcharotone K, Mukherji SK |title=Reassessing the Anatomic Origin of the Juvenile Nasopharyngeal Angiofibroma |journal=J Comput Assist Tomogr |volume=41 |issue=4 |pages=559–564 |date=2017 |pmid=28632604 |doi=10.1097/RCT.0000000000000566 |url=}}</ref>
:*Polypoid fibrous mass
**Tan to purple-red, rubbery-firm unencapsulated polypoid [[fibrous]] mass  
:*Bleeding on manipulation
**Bleeding on [[biopsy]]
*On microscopic histopathological analysis, characteristic findings of nasopharyngeal angiofibroma, include:
**Spongy cut surface
:*Fibroblastic cells with plump (near cuboidal) nuclei
**[[Sessile]] or [[pedunculated]] [[tumor]]
:*Fibrous stroma
 
:*Abundant capillaries
*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==
==Causes==
* There are no known causes of nasopharyngeal angiofibroma.
* The cause of Nasopharyngeal angiofibroma has not been identified, but it may be caused by [[hormonal]] effect.<ref name="pmid25384380">{{cite journal |vauthors=Liu Z, Wang J, Wang H, Wang D, Hu L, Liu Q, Sun X |title=Hormonal receptors and vascular endothelial growth factor in juvenile nasopharyngeal angiofibroma: immunohistochemical and tissue microarray analysis |journal=Acta Otolaryngol. |volume=135 |issue=1 |pages=51–7 |date=January 2015 |pmid=25384380 |doi=10.3109/00016489.2014.952774 |url=}}</ref>


==Differentiating Nasopharyngeal Angiofibroma from Other Diseases==
* Nasopharyngeal angiofibroma express higher levels of [[vascular endothelial growth factor]] (VEGF) and [[hormone]] [[receptors]].
*Nasopharyngeal angiofibroma must be differentiated from other diseases that cause epistaxis, unilateral nasal obstruction, and rhinorrhea, such as:
 
:* Antro-choanal polyp  
==Differentiating Nasopharyngeal angiofibroma from Other Diseases==
Nasopharyngeal angiofibroma must be differentiated from other diseases that cause [[epistaxis]], unilateral [[nasal]] obstruction, and [[rhinorrhea]], such as:<ref name="pmid30038985">{{cite journal |vauthors=Alimli AG, Ucar M, Oztunali C, Akkan K, Boyunaga O, Damar C, Derinkuyu B, Tokgöz N |title=Juvenile Nasopharyngeal Angiofibroma: Magnetic Resonance Imaging Findings |journal=J Belg Soc Radiol |volume=100 |issue=1 |pages=63 |date=June 2016 |pmid=30038985 |pmc=5854277 |doi=10.5334/jbr-btr.1090 |url=}}</ref>
:* Sinonasal [[polyp]]
:* [[Rhinosporidiosis]]  
:* [[Rhinosporidiosis]]  
:* [[Chordoma]]
:* [[Chordoma]]
:* Nasopharanageal cyst
:* [[Nasopharyngeal]] [[cyst]]
:* [[Pyogenic granuloma]]
:* [[Pyogenic granuloma]]
:* [[Nasopharyngeal carcinoma]]
:* [[Lymphoma]]
:* [[Rhabdomyosarcoma]]
:* [[Adenoid]] [[hypertrophy]]


==Epidemiology and Demographics==
==Epidemiology and Demographics==
* Nasopharyngeal angiofibroma is a rare tumor.
* [[Incidence]] of juvenile nasopharyngeal angiofibroma is less than 0.5% of head and neck [[Tumor|tumors]]. <ref name="pmid13230032">{{cite journal |vauthors=Gullane PJ, Davidson J, O'Dwyer T, Forte V |title=Juvenile angiofibroma: a review of the literature and a case series report |journal=Laryngoscope |volume=102 |issue=8 |pages=928–33 |date=August 1992 |pmid=1323003 |doi=10.1288/00005537-199208000-00014 |url=}}</ref>
* Nasopharyngeal angiofibroma accounts for 0.05% of all head and neck tumors.
* The [[incidence]] of juvenile nasopharyngeal angiofibroma is 0.6 per 100,000 individuals worldwide.<ref name="pmid182285212">{{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 |date=March 2008 |pmid=18228521 |doi=10.1002/hed.20775 |url=}}</ref>
* The prevalence of nasopharyngeal angiofibroma is approximately 0.4 per 100,000 individuals worldwide.<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>
* Some reports suggest juvenile nasopharyngeal angiofibroma is more common in the Indian subcontinent than in the west.<ref name="pmid17368816">{{cite journal |vauthors=Biswas D, Saha S, Bera SP |title=Relative distribution of the tumours of ear, nose and throat in the paediatric patients |journal=Int. J. Pediatr. Otorhinolaryngol. |volume=71 |issue=5 |pages=801–5 |date=May 2007 |pmid=17368816 |doi=10.1016/j.ijporl.2007.01.021 |url=}}</ref>
* Men are more commonly affected by juvenile nasopharyngeal angiofibroma than women.
 
==Risk Factors==
 
Common [[risk factors]] in the development of nasopharyngeal angiofibroma include:<ref name="pmid18227724">{{cite journal |vauthors=Ponti G, Losi L, Pellacani G, Rossi GB, Presutti L, Mattioli F, Villari D, Wannesson L, Alicandri Ciufelli M, Izzo P, De Rosa M, Marone P, Seidenari S |title=Wnt pathway, angiogenetic and hormonal markers in sporadic and familial adenomatous polyposis-associated juvenile nasopharyngeal angiofibromas (JNA) |journal=Appl. Immunohistochem. Mol. Morphol. |volume=16 |issue=2 |pages=173–8 |date=March 2008 |pmid=18227724 |doi=10.1097/PAI.0b013e31806bee12 |url=}}</ref>
* [[Familial adenomatous polyposis]]
* Male gender
*
 
==Screening==
* There is insufficient evidence to recommend routine [[screening]] for nasopharyngeal angiofibroma.
 
==Natural History, Complications, and Prognosis==
* If left untreated, %9 of patients with nasopharyngeal angiofibroma may progress to develop [[hemorrhage]] and intracranial extension. 
 
* Common complications of nasopharengeal angiofibroma include nasal [[obstruction]] and repeated [[bleeding]].<ref name="pmid28286392">{{cite journal |vauthors=Roy Chowdhury S, Rajkumar K, Deshmukh T |title=Complications of Midface Swing for Management of Juvenile Nasopharyngeal Angiofibroma |journal=J Maxillofac Oral Surg |volume=16 |issue=1 |pages=96–100 |date=March 2017 |pmid=28286392 |pmc=5328877 |doi=10.1007/s12663-016-0947-x |url=}}</ref>
 
* In general prognosis of nasopharyngeal angiofibroma is good.
 
==Diagnosis==
===Diagnostic Study of Choice===
The diagnosis of nasopharyngeal angiofibroma is made when the following findings are met:
* [[Epistaxis]]
* Unilateral nasal [[obstruction]]
* Mass in the [[nasopharynx]]
 
===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, [[epistaxis]] and [[headache]].
* Less common symptoms of Nasopharyngeal angiofibroma include [[conductive hearing loss]], [[diplopia]], [[proptosis]], [[anosmia]], recurrent [[otitis media]] and eye pain. <ref name="pmid15548906" />


===Age===
===Physical Examination===
*Nasopharyngeal angiofibroma is more commonly observed among patients aged 7-19 years.<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>
* Physical examination of patients with Nasopharyngeal angiofibroma is usually remarkable for '''nasal mass and [[rhinorrhea]]'''.<ref name="pmid15548906">{{cite journal |vauthors=Enepekides DJ |title=Recent advances in the treatment of juvenile angiofibroma |journal=Curr Opin Otolaryngol Head Neck Surg |volume=12 |issue=6 |pages=495–9 |date=December 2004 |pmid=15548906 |doi= |url=}}</ref>
*Nasopharyngeal angiofibroma is more commonly observed among  children and adolescents .
* Rare physical examination include:
 
===Gender===
** [[Orbit (anatomy)|Orbital]] mass
*Males are more commonly affected with nasopharyngeal angiofibroma than females.<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>
** [[Proptosis]]
*The male to female ratio for nasopharyngeal angiofibroma is approximately 4 to 1.<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>
** [[Zygomatic]] [[swelling]]
** [[Trismus]]
===Race===
** Decreasing [[vision]]
*There is no racial predilection for nasopharyngeal angiofibroma.
*  
*  
 
===Laboratory Findings===


==Risk Factors==
There are no diagnostic laboratory findings associated with nasopharyngeal angiofibroma.
*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==
===Electrocardiogram===
*The majority of patients with nasopharyngeal angiofibroma are symptomatic at diagnosis.
There are no ECG findings associated with nasopharyngeal angiofibroma.  
*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 ==
===X-ray===
===Diagnostic Criteria===
An x-ray may be helpful in the diagnosis of nasopharyngeal angiofibroma. Findings on an x-ray suggestive of nasopharyngeal angiofibroma include:<ref name="pmid26046027">{{cite journal |vauthors=Ikubor JE, Okolugbo NE, Okhakhu AL |title=Radiological features of juvenile nasopharyngeal angiofibroma |journal=J West Afr Coll Surg |volume=3 |issue=4 |pages=84–91 |date=2013 |pmid=26046027 |pmc=4437236 |doi= |url=}}</ref>
*The diagnosis of nasopharyngeal angiofibroma is made when at least [number] of the following [number] diagnostic criteria are met:
* Nasopharyngeal [[polyp]]
:*Clinical criteria
* Antral sign or Holman-Miller sign: Forward bowing of the [[posterior]] wall of the [[maxillary sinus]]  
::*Young patient
* [[Maxillary sinus]] opacification
::*[[Epistaxis causes|Epistaxis]]  
* Bony margin may be eroded
:*Positive physical exam
::*A smooth submucosal mass in the posterior nasal cavity
:*Positive imaging finding: visualisation of a nasopharyngeal mass


=== Symptoms ===
===Echocardiography or Ultrasound===
*Common symptoms of nasopharyngeal angiofibroma, may include:
* There are no echocardiography/ultrasound findings associated with nasopharyngeal angiofibroma.
:*Epistaxis or blood-tinged nasal discharge
:*Unilateral nasal obstruction
:*[[Rhinorrhea]]
:*[[Hearing loss]]
:*[[Diplopia]]
:*Rarely [[Anosmia causes|anosmia]]
:*[[Eye pain]]


=== Physical Examination ===
===CT scan===
*Patients with nasopharyngeal angiofibroma usually are well-appearing.
CT scan demonstrate the extent of the tumor. Findings on CT scan suggestive of nasopharengeal angiofibroma include:<ref name="pmid23878770">{{cite journal |vauthors=Mishra S, Praveena NM, Panigrahi RG, Gupta YM |title=Imaging in the diagnosis of juvenile nasopharyngeal angiofibroma |journal=J Clin Imaging Sci |volume=3 |issue=Suppl 1 |pages=1 |date=2013 |pmid=23878770 |pmc=3716018 |doi=10.4103/2156-7514.109469 |url=}}</ref>
*Physical examination  may be remarkable for:
* Extension to the [[sphenoid sinus]]
:*A smooth submucosal mass in the posterior nasal cavity
* Erosion of the greater sphenoidal wing
* invasion of the [[pterygomaxillary]] and infratemporal fossae


=== Laboratory Findings ===
===MRI===
*There are no specific laboratory findings associated with the diagnosis of nasopharyngeal angiofibroma.
Cronal MRI demonstrate the extent of the tumor especially in intracranial involvement. <ref name="pmid238787702">{{cite journal |vauthors=Mishra S, Praveena NM, Panigrahi RG, Gupta YM |title=Imaging in the diagnosis of juvenile nasopharyngeal angiofibroma |journal=J Clin Imaging Sci |volume=3 |issue=Suppl 1 |pages=1 |date=2013 |pmid=23878770 |pmc=3716018 |doi=10.4103/2156-7514.109469 |url=}}</ref>
* Findings on MRI suggestive of nasopharengeal angiofibroma include:<ref name="pmid300389852">{{cite journal |vauthors=Alimli AG, Ucar M, Oztunali C, Akkan K, Boyunaga O, Damar C, Derinkuyu B, Tokgöz N |title=Juvenile Nasopharyngeal Angiofibroma: Magnetic Resonance Imaging Findings |journal=J Belg Soc Radiol |volume=100 |issue=1 |pages=63 |date=June 2016 |pmid=30038985 |pmc=5854277 |doi=10.5334/jbr-btr.1090 |url=}}</ref>
===Imaging Findings===
** Largely isointense to muscle on T1-weighted images
*Computed tomography is the imaging modality of choice for nasopharyngeal angiofibroma.
** Hyperintense on T2-weighted images
*On conventional radiography, findings of nasopharyngeal angiofibroma, include:  
** Internal signal-void regions
:*Visualisation of a nasopharyngeal mass
** Intense enhancement after [[intravenous]] (IV) [[contrast]] injection
:*Opacification of the sphenoid sinus
The images below demonstrate findings of nasopharyngeal angiofibroma.  
:*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.  
<gallery>
<gallery>
Image:Nasopharyngeal angiofibroma 001.jpg| Nasopharyngeal angiofibroma. Skull base invasion[http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology]
Image:Nasopharyngeal angiofibroma 001.jpg| Nasopharyngeal angiofibroma. Skull base invasion[http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology]
Line 141: Line 153:
</gallery>
</gallery>


=== Other Diagnostic Studies ===
 
*Nasopharyngeal angiofibroma may also be diagnosed using nasal [[endoscopy]].  
 
===Other Imaging Findings===
== Treatment ==
 
[[Digital subtraction angiography|DSA]] ([[digital subtraction angiography]]) of [[carotid artery]] is helpful in the diagnosis extension of nasopharengeal angiofibroma and feeding [[vessel]].<ref name="pmid16775738">{{cite journal |vauthors=Giavroglou C, Constantinidis J, Triaridis S, Daniilidis J, Dimitriadis A |title=[Angiographic evaluation and embolization of juvenile nasopharyngeal angiofibroma] |language=German |journal=HNO |volume=55 |issue=1 |pages=36–41 |date=January 2007 |pmid=16775738 |doi=10.1007/s00106-006-1410-y |url=}}</ref>
 
===Other Diagnostic Studies===
Other diagnostic studies for nasopharengeal angiofibroma include nasal endoscopy, which demonstrates hypervascularized large, lobulated mass behind the middle turbinate.<ref name="pmid27601836">{{cite journal |vauthors=Makhasana JA, Kulkarni MA, Vaze S, Shroff AS |title=Juvenile nasopharyngeal angiofibroma |journal=J Oral Maxillofac Pathol |volume=20 |issue=2 |pages=330 |date=2016 |pmid=27601836 |pmc=4989574 |doi=10.4103/0973-029X.185908 |url=}}</ref><ref name="pmid221641852">{{cite journal |vauthors=Nicolai P, Schreiber A, Bolzoni Villaret A |title=Juvenile angiofibroma: evolution of management |journal=Int J Pediatr |volume=2012 |issue= |pages=412545 |date=2012 |pmid=22164185 |doi=10.1155/2012/412545 |url=}}</ref>
 
=== Medical Therapy ===
=== Medical Therapy ===
*Medical therapy for nasopharyngeal angiofibroma is divided into 2 categories:<ref name="pmid22164185">{{cite journal |vauthors=Nicolai P, Schreiber A, Bolzoni Villaret A |title=Juvenile angiofibroma: evolution of management |journal=Int J Pediatr |volume=2012 |issue= |pages=412545 |year=2012 |pmid=22164185 |pmc=3228400 |doi=10.1155/2012/412545 |url=}}</ref>
The mainstay of treatment for nasopharyngeal angiofibroma is [[surgical resection]].
:*[[Hormonal therapy]]
* Medical therapies for nasopharyngeal angiofibroma include: <ref name="pmid155489062">{{cite journal |vauthors=Enepekides DJ |title=Recent advances in the treatment of juvenile angiofibroma |journal=Curr Opin Otolaryngol Head Neck Surg |volume=12 |issue=6 |pages=495–9 |date=December 2004 |pmid=15548906 |doi= |url=}}</ref>
:*[[Radiotherapy]]
** [[Hormones]] ([[estrogen]], [[testosterone]])
*Hormonal therapy for nasopharyngeal angiofibroma, includes:<ref name="pmid22164185">{{cite journal |vauthors=Nicolai P, Schreiber A, Bolzoni Villaret A |title=Juvenile angiofibroma: evolution of management |journal=Int J Pediatr |volume=2012 |issue= |pages=412545 |year=2012 |pmid=22164185 |pmc=3228400 |doi=10.1155/2012/412545 |url=}}</ref>
** [[Radiation therapy]]
:*[[Flutamide]]
** [[Chemotherapy]]
*Medical treatment is usually given before surgery to reduce the blood loss
** [[Embolization]]
*Radiotherapy for nasopharyngeal angiofibroma, include:
:*Stereotactic radiotherapy


=== Surgery ===
===Surgery===
*Surgery is the mainstay of therapy for nasopharyngeal angiofibroma.<ref name="pmid22164185">{{cite journal |vauthors=Nicolai P, Schreiber A, Bolzoni Villaret A |title=Juvenile angiofibroma: evolution of management |journal=Int J Pediatr |volume=2012 |issue= |pages=412545 |year=2012 |pmid=22164185 |pmc=3228400 |doi=10.1155/2012/412545 |url=}}</ref>
[[Arteriography]] followed by preoperative [[embolization]] and [[surgical resection]] is the mainstay of treatment for nasopharyngeal angiofibroma. <ref name="pmid221641854">{{cite journal |vauthors=Nicolai P, Schreiber A, Bolzoni Villaret A |title=Juvenile angiofibroma: evolution of management |journal=Int J Pediatr |volume=2012 |issue= |pages=412545 |date=2012 |pmid=22164185 |pmc=3228400 |doi=10.1155/2012/412545 |url=}}</ref>
*Preoperative embolization is highly suggested before surgery.
* Surgical approaches for nasopharyngeal angiofibroma include: <ref name="pmid221641853">{{cite journal |vauthors=Nicolai P, Schreiber A, Bolzoni Villaret A |title=Juvenile angiofibroma: evolution of management |journal=Int J Pediatr |volume=2012 |issue= |pages=412545 |date=2012 |pmid=22164185 |doi=10.1155/2012/412545 |url=}}</ref>
*Surgical approach for nasopharyngeal angiofibroma will depend on the stage.<ref name="pmid22164185">{{cite journal |vauthors=Nicolai P, Schreiber A, Bolzoni Villaret A |title=Juvenile angiofibroma: evolution of management |journal=Int J Pediatr |volume=2012 |issue= |pages=412545 |year=2012 |pmid=22164185 |pmc=3228400 |doi=10.1155/2012/412545 |url=}}</ref>
** Transpalatal
:*The treatment of choice  for early stage for nasopharyngeal angiofibroma is intranasal endoscopic surgery. Lateral rhinotomy is the preferred surgical approach.
** Transmaxillary
:*The treatment of choice  for late stage for nasopharyngeal angiofibroma include the infratemporal fossa approach, and the mid-facial degloving approach.
** Lateral rhinotomy
** Mid-facial degloving
** Extended sublabial Denker
** Le Fort type I [[osteotomy]]
** [[Endoscopic surgery]]


=== Prevention ===
===Primary Prevention===
*There are no primary preventive measures available for nasopharyngeal angiofibroma.
There are no established measures for the primary prevention of 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.  
 
===Secondary Prevention===
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.


==References==
==References==
{{Reflist|2}}
{{reflist|2}}
 
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Latest revision as of 15:00, 27 February 2019


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mahda Alihashemi M.D. [2], Maria Fernanda Villarreal, M.D. [3]

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

Overview

Nasopharyngeal angiofibroma is a benign, vascular, polypoid fibrous mass in located in the the posterolateral wall of the nasal cavity. It was first described by Hippocrates, a Greek physician, in the 5th century BC. Nasopharyngeal angiofibroma may be classified according to Fisch classification into 4 groups. Nasopharyngeal angiofibroma is a histologically benign tumor. This tumor is located in the posterolateral wall of the nasal cavity. On gross pathology, characteristic findings of nasopharyngeal angiofibroma include tan to purple-red, rubbery-firm unencapsulated polypoid fibrous mass. Fibroblastic cells with plump (near cuboidal) nuclei and abundant capillaries are seen on microscopic histopathological analysis. The cause of Nasopharyngeal angiofibroma has not been identified, but it may be caused by hormonal effect. Incidence of juvenile nasopharyngeal angiofibroma is less than 0.5% of head and neck tumors. If left untreated, %9 of patients with nasopharyngeal angiofibroma may progress to develop hemorrhage and intracranial extension. The hallmark of Nasopharyngeal angiofibroma is the classic triad of epistaxis, unilateral nasal obstruction and a mass in the nasopharynx. Physical examination of patients with Nasopharyngeal angiofibroma is usually remarkable for nasal mass and rhinorrhea. Findings on an x-ray suggestive of nasopharyngeal angiofibroma include nasopharyngeal polyp and Antral sign or Holman-Miller sign (Forward bowing of the posterior wall of the maxillary sinus). CT scan and MRI demonstrate the extent of the tumor. The mainstay of treatment for nasopharyngeal angiofibroma is surgical resection.

Historical Perspective

  • Nasopharyngeal angiofibroma was first described by Hippocrates, a Greek physician, in the 5th century BC.[1]
  • In 1940, Friedberg was the first to use the term angiofibroma.
  • The first endoscopic resection of nasopharyngeal angiofibroma was done in 1996.

Classification

Nasopharyngeal angiofibroma may be classified according to Fisch classification into 4 groups: [2]

Pathophysiology

  • Nasopharyngeal angiofibroma is a histologically benign tumor.
  • Genetic alterations associated with the development of nasopharyngeal angiofibroma include:[4]
  • On gross pathology, characteristic findings of nasopharyngeal angiofibroma include:
  • On microscopic histopathological analysis, characteristic findings of nasopharyngeal angiofibroma include:

Causes

  • The cause of Nasopharyngeal angiofibroma has not been identified, but it may be caused by hormonal effect.[6]

Differentiating Nasopharyngeal angiofibroma from Other Diseases

Nasopharyngeal angiofibroma must be differentiated from other diseases that cause epistaxis, unilateral nasal obstruction, and rhinorrhea, such as:[7]

Epidemiology and Demographics

  • Incidence of juvenile nasopharyngeal angiofibroma is less than 0.5% of head and neck tumors. [8]
  • The incidence of juvenile nasopharyngeal angiofibroma is 0.6 per 100,000 individuals worldwide.[9]
  • Some reports suggest juvenile nasopharyngeal angiofibroma is more common in the Indian subcontinent than in the west.[10]
  • Men are more commonly affected by juvenile nasopharyngeal angiofibroma than women.

Risk Factors

Common risk factors in the development of nasopharyngeal angiofibroma include:[11]

Screening

  • There is insufficient evidence to recommend routine screening for nasopharyngeal angiofibroma.

Natural History, Complications, and Prognosis

  • If left untreated, %9 of patients with nasopharyngeal angiofibroma may progress to develop hemorrhage and intracranial extension.
  • In general prognosis of nasopharyngeal angiofibroma is good.

Diagnosis

Diagnostic Study of Choice

The diagnosis of nasopharyngeal angiofibroma is made when the following findings are met:

History and Symptoms

Physical Examination

  • Physical examination of patients with Nasopharyngeal angiofibroma is usually remarkable for nasal mass and rhinorrhea.[13]
  • Rare physical examination include:

Laboratory Findings

There are no diagnostic laboratory findings associated with nasopharyngeal angiofibroma.

Electrocardiogram

There are no ECG findings associated with nasopharyngeal angiofibroma.

X-ray

An x-ray may be helpful in the diagnosis of nasopharyngeal angiofibroma. Findings on an x-ray suggestive of nasopharyngeal angiofibroma include:[14]

Echocardiography or Ultrasound

  • There are no echocardiography/ultrasound findings associated with nasopharyngeal angiofibroma.

CT scan

CT scan demonstrate the extent of the tumor. Findings on CT scan suggestive of nasopharengeal angiofibroma include:[15]

MRI

Cronal MRI demonstrate the extent of the tumor especially in intracranial involvement. [16]

  • Findings on MRI suggestive of nasopharengeal angiofibroma include:[17]
    • Largely isointense to muscle on T1-weighted images
    • Hyperintense on T2-weighted images
    • Internal signal-void regions
    • Intense enhancement after intravenous (IV) contrast injection

The images below demonstrate findings of nasopharyngeal angiofibroma.


Other Imaging Findings

DSA (digital subtraction angiography) of carotid artery is helpful in the diagnosis extension of nasopharengeal angiofibroma and feeding vessel.[18]

Other Diagnostic Studies

Other diagnostic studies for nasopharengeal angiofibroma include nasal endoscopy, which demonstrates hypervascularized large, lobulated mass behind the middle turbinate.[19][20]

Medical Therapy

The mainstay of treatment for nasopharyngeal angiofibroma is surgical resection.

Surgery

Arteriography followed by preoperative embolization and surgical resection is the mainstay of treatment for nasopharyngeal angiofibroma. [22]

  • Surgical approaches for nasopharyngeal angiofibroma include: [23]
    • Transpalatal
    • Transmaxillary
    • Lateral rhinotomy
    • Mid-facial degloving
    • Extended sublabial Denker
    • Le Fort type I osteotomy
    • Endoscopic surgery

Primary Prevention

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

Secondary Prevention

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.

References

  1. ↑ Moorthy PN, Ranganatha Reddy B, Qaiyum HA, Madhira S, Kolloju S (October 2010). "Management of juvenile nasopharyngeal angiofibroma: a five year retrospective study". Indian J Otolaryngol Head Neck Surg. 62 (4): 390–4. doi:10.1007/s12070-010-0097-2. PMC 3266082. PMID 22319699.
  2. ↑ Martins MB, de Lima FV, Mendonça CA, de Jesus EP, Santos AC, Barreto VM, Santos RC (January 2013). "Nasopharyngeal angiofibroma: Our experience and literature review". Int Arch Otorhinolaryngol. 17 (1): 14–9. doi:10.7162/S1809-97772013000100003. PMC 4423317. PMID 25991988.
  3. ↑ Pryor SG, Moore EJ, Kasperbauer JL (July 2005). "Endoscopic versus traditional approaches for excision of juvenile nasopharyngeal angiofibroma". Laryngoscope. 115 (7): 1201–7. doi:10.1097/01.MLG.0000162655.96247.66. PMID 15995507.
  4. ↑
  5. ↑ McKnight CD, Parmar HA, Watcharotone K, Mukherji SK (2017). "Reassessing the Anatomic Origin of the Juvenile Nasopharyngeal Angiofibroma". J Comput Assist Tomogr. 41 (4): 559–564. doi:10.1097/RCT.0000000000000566. PMID 28632604.
  6. ↑ Liu Z, Wang J, Wang H, Wang D, Hu L, Liu Q, Sun X (January 2015). "Hormonal receptors and vascular endothelial growth factor in juvenile nasopharyngeal angiofibroma: immunohistochemical and tissue microarray analysis". Acta Otolaryngol. 135 (1): 51–7. doi:10.3109/00016489.2014.952774. PMID 25384380.
  7. ↑ Alimli AG, Ucar M, Oztunali C, Akkan K, Boyunaga O, Damar C, Derinkuyu B, Tokgöz N (June 2016). "Juvenile Nasopharyngeal Angiofibroma: Magnetic Resonance Imaging Findings". J Belg Soc Radiol. 100 (1): 63. doi:10.5334/jbr-btr.1090. PMC 5854277. PMID 30038985.
  8. ↑ Gullane PJ, Davidson J, O'Dwyer T, Forte V (August 1992). "Juvenile angiofibroma: a review of the literature and a case series report". Laryngoscope. 102 (8): 928–33. doi:10.1288/00005537-199208000-00014. PMID 1323003.
  9. ↑ Coutinho-Camillo CM, Brentani MM, Nagai MA (March 2008). "Genetic alterations in juvenile nasopharyngeal angiofibromas". Head Neck. 30 (3): 390–400. doi:10.1002/hed.20775. PMID 18228521.
  10. ↑ Biswas D, Saha S, Bera SP (May 2007). "Relative distribution of the tumours of ear, nose and throat in the paediatric patients". Int. J. Pediatr. Otorhinolaryngol. 71 (5): 801–5. doi:10.1016/j.ijporl.2007.01.021. PMID 17368816.
  11. ↑ Ponti G, Losi L, Pellacani G, Rossi GB, Presutti L, Mattioli F, Villari D, Wannesson L, Alicandri Ciufelli M, Izzo P, De Rosa M, Marone P, Seidenari S (March 2008). "Wnt pathway, angiogenetic and hormonal markers in sporadic and familial adenomatous polyposis-associated juvenile nasopharyngeal angiofibromas (JNA)". Appl. Immunohistochem. Mol. Morphol. 16 (2): 173–8. doi:10.1097/PAI.0b013e31806bee12. PMID 18227724.
  12. ↑ Roy Chowdhury S, Rajkumar K, Deshmukh T (March 2017). "Complications of Midface Swing for Management of Juvenile Nasopharyngeal Angiofibroma". J Maxillofac Oral Surg. 16 (1): 96–100. doi:10.1007/s12663-016-0947-x. PMC 5328877. PMID 28286392.
  13. ↑ 13.0 13.1 Enepekides DJ (December 2004). "Recent advances in the treatment of juvenile angiofibroma". Curr Opin Otolaryngol Head Neck Surg. 12 (6): 495–9. PMID 15548906.
  14. ↑ Ikubor JE, Okolugbo NE, Okhakhu AL (2013). "Radiological features of juvenile nasopharyngeal angiofibroma". J West Afr Coll Surg. 3 (4): 84–91. PMC 4437236. PMID 26046027.
  15. ↑ Mishra S, Praveena NM, Panigrahi RG, Gupta YM (2013). "Imaging in the diagnosis of juvenile nasopharyngeal angiofibroma". J Clin Imaging Sci. 3 (Suppl 1): 1. doi:10.4103/2156-7514.109469. PMC 3716018. PMID 23878770.
  16. ↑ Mishra S, Praveena NM, Panigrahi RG, Gupta YM (2013). "Imaging in the diagnosis of juvenile nasopharyngeal angiofibroma". J Clin Imaging Sci. 3 (Suppl 1): 1. doi:10.4103/2156-7514.109469. PMC 3716018. PMID 23878770.
  17. ↑ Alimli AG, Ucar M, Oztunali C, Akkan K, Boyunaga O, Damar C, Derinkuyu B, Tokgöz N (June 2016). "Juvenile Nasopharyngeal Angiofibroma: Magnetic Resonance Imaging Findings". J Belg Soc Radiol. 100 (1): 63. doi:10.5334/jbr-btr.1090. PMC 5854277. PMID 30038985.
  18. ↑ Giavroglou C, Constantinidis J, Triaridis S, Daniilidis J, Dimitriadis A (January 2007). "[Angiographic evaluation and embolization of juvenile nasopharyngeal angiofibroma]". HNO (in German). 55 (1): 36–41. doi:10.1007/s00106-006-1410-y. PMID 16775738.
  19. ↑ Makhasana JA, Kulkarni MA, Vaze S, Shroff AS (2016). "Juvenile nasopharyngeal angiofibroma". J Oral Maxillofac Pathol. 20 (2): 330. doi:10.4103/0973-029X.185908. PMC 4989574. PMID 27601836.
  20. ↑ Nicolai P, Schreiber A, Bolzoni Villaret A (2012). "Juvenile angiofibroma: evolution of management". Int J Pediatr. 2012: 412545. doi:10.1155/2012/412545. PMID 22164185.
  21. ↑ Enepekides DJ (December 2004). "Recent advances in the treatment of juvenile angiofibroma". Curr Opin Otolaryngol Head Neck Surg. 12 (6): 495–9. PMID 15548906.
  22. ↑ Nicolai P, Schreiber A, Bolzoni Villaret A (2012). "Juvenile angiofibroma: evolution of management". Int J Pediatr. 2012: 412545. doi:10.1155/2012/412545. PMC 3228400. PMID 22164185.
  23. ↑ Nicolai P, Schreiber A, Bolzoni Villaret A (2012). "Juvenile angiofibroma: evolution of management". Int J Pediatr. 2012: 412545. doi:10.1155/2012/412545. PMID 22164185.


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