eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Pediatric Otolaryngology

Juvenile Nasopharyngeal Angiofibroma: Workup

Author: Ted L Tewfik, MD, FRCS(C), Professor, Department of Otolaryngology, Director of Continuing Medical Education of Otolaryngology, McGill University Medical School; Director, Director of Professional Affairs of Otolaryngology, Department of Otolaryngology, Montreal Children's Hospital; Senior Staff, Montreal General Hospital and Royal Victoria Hospital
Coauthor(s): Mohammed A Al Garni, MBBS, Fellow, Department of Otolaryngology, McGill University, Canada
Contributor Information and Disclosures

Updated: Aug 27, 2009

Workup

Imaging Studies

  • Plain radiography
    • View of the sinuses may demonstrate nasopharyngeal polyp.
    • Bowing of the posterior wall of the maxillary sinus and maxillary sinus opacification is very suggestive of juvenile nasopharyngeal angiofibroma (JNA).
    • Newer radiographic modalities have surpassed plain films in usefulness.
  • CT scanning
    • Images demonstrate extent of the tumor (see Images 1-2).
    • Extension to the sphenoid sinus, erosion of the greater sphenoidal wing, or invasion of the pterygomaxillary and infratemporal fossae is usually visualized.

Coronal CT scan of the lesion filling the left na...

Coronal CT scan of the lesion filling the left nasal cavity and ethmoid sinuses, blocking the maxillary sinus and deviating the nasal septum to the right side.

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Coronal CT scan of the lesion filling the left na...

Coronal CT scan of the lesion filling the left nasal cavity and ethmoid sinuses, blocking the maxillary sinus and deviating the nasal septum to the right side.


Axial CT scan of lesion involving the right nasal...

Axial CT scan of lesion involving the right nasal cavity and paranasal sinuses. Courtesy of J Otolaryngol 1999;28:145.

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Axial CT scan of lesion involving the right nasal...

Axial CT scan of lesion involving the right nasal cavity and paranasal sinuses. Courtesy of J Otolaryngol 1999;28:145.


  • Magnetic resonance imaging (MRI) is indicated to delineate and define the extent of the tumor (see Image 3), especially in cases of intracranial involvement.

Coronal MRI scan showing extension of the lesion ...

Coronal MRI scan showing extension of the lesion to the cavernous sinus. Courtesy of J Otolaryngol 1999;28:145.

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Coronal MRI scan showing extension of the lesion ...

Coronal MRI scan showing extension of the lesion to the cavernous sinus. Courtesy of J Otolaryngol 1999;28:145.


  • Angiography shows the branches of the external carotid system to be the primary feeders (94%).
    • The main supply comes from the internal maxillary artery, but ascending pharyngeal or vidian arteries may contribute to the blood supply.
    • Unnamed branches from the internal carotid artery contribute to vascularity in rare instances.
    • An angiofibroma before embolization can be seen in Image 4. An angiofibroma after embolization can be seen in Image 5.

Angiogram depicting angiofibroma before embolizat...

Angiogram depicting angiofibroma before embolization. Courtesy of J Otolaryngol 1999;28:145.

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Angiogram depicting angiofibroma before embolizat...

Angiogram depicting angiofibroma before embolization. Courtesy of J Otolaryngol 1999;28:145.


Angiogram depicting angiofibroma after embolizati...

Angiogram depicting angiofibroma after embolization. Courtesy of J Otolaryngol 1999;28:145.

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Angiogram depicting angiofibroma after embolizati...

Angiogram depicting angiofibroma after embolization. Courtesy of J Otolaryngol 1999;28:145.


Histologic Findings

On gross examination, the tumor is usually sessile, lobulated, rubbery, and red-pink to tan-gray in appearance. In rare cases, the tumor is polypoid or pedunculated.

Nasopharyngeal angiofibroma is usually encapsulated and composed of vascular tissue and fibrous stroma with coarse or fine collagen fibers. Vessels are thin-walled, lack elastic fibers, have absent or incomplete smooth muscle, and vary in appearance from stellate or staghorn to barely conspicuous because of stromal compression. Stromal cells have plump nuclei and tend to radiate around the vessels. An abundance of mast cells in the stroma and a lack of other inflammatory cells exist. Localized areas of myxomatous degeneration may be observed in the stroma.

When examined under electron microscope, stromal cells are mostly fibroblasts and show intensive immunostaining for vimentin. However, myofibroblasts may occur focally in connection with fibrotic areas and are characterized by the coexpression of vimentin and smooth muscle actin.

Staging

Different staging systems exist for nasopharyngeal angiofibroma. The 2 most commonly used are those of Sessions and Fisch.

  • Classification according to Sessions
    • Stage IA - Tumor limited to posterior nares and/or nasopharyngeal vault
    • Stage IB - Tumor involving posterior nares and/or nasopharyngeal vault with involvement of at least 1 paranasal sinus
    • Stage IIA - Minimal lateral extension into pterygomaxillary fossa
    • Stage IIB - Full occupation of pterygomaxillary fossa with or without superior erosion of orbital bones
    • Stage IIIA - Erosion of skull base (ie, middle cranial fossa/pterygoid base); minimal intracranial extension
    • Stage IIIB - Extensive intracranial extension with or without extension into cavernous sinus
  • Classification according to Fisch
    • Stage I - Tumors limited to nasal cavity, nasopharynx with no bony destruction
    • Stage II - Tumors invading pterygomaxillary fossa, paranasal sinuses with bony destruction
    • Stage III - Tumors invading infratemporal fossa, orbit and/or parasellar region remaining lateral to cavernous sinus
    • Stage IV - Tumors invading cavernous sinus, optic chiasmal region, and/or pituitary fossa

More on Juvenile Nasopharyngeal Angiofibroma

Overview: Juvenile Nasopharyngeal Angiofibroma
Workup: Juvenile Nasopharyngeal Angiofibroma
Treatment: Juvenile Nasopharyngeal Angiofibroma
Follow-up: Juvenile Nasopharyngeal Angiofibroma
Multimedia: Juvenile Nasopharyngeal Angiofibroma
References
Further Reading
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References

  1. Schuon R, Brieger J, Heinrich UR, Roth Y, Szyfter W, Mann WJ. Immunohistochemical analysis of growth mechanisms in juvenile nasopharyngeal angiofibroma. Eur Arch Otorhinolaryngol. Apr 2007;264(4):389-94. [Medline].

  2. Hackman T, Snyderman CH, Carrau R, Vescan A, Kassam A. Juvenile nasopharyngeal angiofibroma: The expanded endonasal approach. Am J Rhinol Allergy. Jan-Feb 2009;23(1):95-9. [Medline].

  3. Tang IP, Shashinder S, Gopala Krishnan G, Narayanan P. Juvenile nasopharyngeal angiofibroma in a tertiary centre: ten-year experience. Singapore Med J. Mar 2009;50(3):261-4. [Medline].

  4. Makek MS, Andrews JC, Fisch U. Malignant transformation of a nasopharyngeal angiofibroma. Laryngoscope. Oct 1989;99(10 Pt 1):1088-92. [Medline].

  5. Baguley C, Sandhu G, O'Donnell J, Howard D. Consumptive coagulopathy complicating juvenile angiofibroma. J Laryngol Otol. Nov 2004;118(11):835-9. [Medline].

  6. Beham A, Kainz J, Stammberger H, Aubock L, Beham-Schmid C. Immunohistochemical and electron microscopical characterization of stromal cells in nasopharyngeal angiofibromas. Eur Arch Otorhinolaryngol. 1997;254(4):196-9. [Medline].

  7. Beriwal S, Eidelman A, Micaily B. Three-dimensional conformal radiotherapy for treatment of extensive juvenile angiofibroma: report on two cases. ORL J Otorhinolaryngol Relat Spec. Jul-Aug 2003;65(4):238-41. [Medline].

  8. Browne JD, Messner AH. Lateral orbital/anterior midfacial degloving approach for nasopharyngeal angiofibromas with cavernous sinus extensions. Skull Base Surg. 1994;4:232-8.

  9. De Vincentiis M, Gallo A, Minni A, Torri E, Tomassi R, Della Rocca C. [Preoperative embolization in the treatment protocol for rhinopharyngeal angiofibroma: comparison of the effectiveness of various materials]. Acta Otorhinolaryngol Ital. Jun 1997;17(3):225-32. [Medline].

  10. Fisch U. The infratemporal fossa approach for nasopharyngeal tumors. Laryngoscope. Jan 1983;93(1):36-44. [Medline].

  11. Goldsmith AJ. Transpalatal approach to the nasopharynx. 1999;10:98-100.

  12. Haines SJ, Duval AJ 3rd. Transzygomatic and palatal excision of juvenile nasopharyngeal angiofibroma with intracranial extension: the surgical procedure. In: Sekhar LN, Janecka IP, eds. Surgery of Cranial Base Tumors. NY: Raven Press; 1993:477-80.

  13. Mair EA, Battiata A, Casler JD. Endoscopic laser-assisted excision of juvenile nasopharyngeal angiofibromas. Arch Otolaryngol Head Neck Surg. Apr 2003;129(4):454-9. [Medline].

  14. Raveh J, Turk JB, Ladrach K, et al. Extended anterior subcranial approach for skull base tumors: long-term results. J Neurosurg. Jun 1995;82(6):1002-10. [Medline].

  15. Schick B, Veldung B, Wemmert S, et al. p53 and Her-2/neu in juvenile angiofibromas. Oncol Rep. Mar 2005;13(3):453-7. [Medline].

  16. Shaheen OH. Angiofibroma. In: John NG, ed. Scott-Brown's Otolaryngology. Vol 6. London: Butterworth-Heinemann; 1987:291-6.

  17. Tewfik TL, Tan AK, al Noury K, et al. Juvenile nasopharyngeal angiofibroma. J Otolaryngol. Jun 1999;28(3):145-51. [Medline].

  18. Tyagi I, Syal R, Goyal A. Recurrent and residual juvenile angiofibromas. J Laryngol Otol. May 2007;121(5):460-7. [Medline].

  19. Wenig BM. Atlas of Head and Neck Pathology. Philadelphia, Pa: WB Saunders Co; 1993:145-6.

  20. Windfuhr JP, Remmert S. Extranasopharyngeal angiofibroma: etiology, incidence and management. Acta Otolaryngol. Oct 2004;124(8):880-9. [Medline].

  21. Wormald PJ, Van Hasselt A. Endoscopic removal of juvenile angiofibromas. Otolaryngol Head Neck Surg. Dec 2003;129(6):684-91. [Medline].

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Further Reading

Clinical guidelines

 Facilities and equipment for the care of pediatric patients in a community hospital.
American Academy of Pediatrics - Medical Specialty Society.  2003 May.  3 pages.  NGC:003056

ACR Appropriateness Criteria® orbits, vision, and visual loss.
American College of Radiology - Medical Specialty Society.  1999 (revised 2006).  9 pages.  NGC:005122

Clinical trials

The Effect of Packing in Post Operative Management of FESS

A Prospective Randomised Controlled Trial of Management of Recurrent Nosebleeds in Children


Related eMedicine topics

 Skull Base Tumors

Epistaxis

Perineural Spread of Tumor Along the Fifth and Seventh Cranial Nerves

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Keywords

juvenile nasopharyngeal angiofibroma, JNA, angiofibroma, nasopharyngeal fibroma, nasopharyngeal angiofibroma, bleeding fibroma of adolescence, fibroangioma, benign tumor, nonchromaffin paraganglionic cells of the terminal branches of the maxillary artery, extranasopharyngeal angiofibroma, nasal mass, orbital mass, proptosis, otitis due to eustachian tube blockage, zygomatic swelling, nonchromaffin paraganglionic cells of the terminal branches of the maxillary artery, tumor of the nasopharynx

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Contributor Information and Disclosures

Author

Ted L Tewfik, MD, FRCS(C), Professor, Department of Otolaryngology, Director of Continuing Medical Education of Otolaryngology, McGill University Medical School; Director, Director of Professional Affairs of Otolaryngology, Department of Otolaryngology, Montreal Children's Hospital; Senior Staff, Montreal General Hospital and Royal Victoria Hospital
Ted L Tewfik, MD, FRCS(C) is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Society of Pediatric Otolaryngology, Canadian Medical Association, Canadian Society of Otolaryngology-Head & Neck Surgery, Quebec Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Coauthor(s)

Mohammed A Al Garni, MBBS, Fellow, Department of Otolaryngology, McGill University, Canada
Disclosure: Nothing to disclose.

Medical Editor

Ari J Goldsmith, MD, Chief of Pediatric Otolaryngology, Long Island College Hospital; Associate Professor, Department of Otolaryngology, Division of Pediatric Otolaryngology, State University of New York Downstate Medical Center
Ari J Goldsmith, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Medical Association, and Medical Society of the State of New York
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Gregory C Allen, MD, Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine
Gregory C Allen, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Cleft Palate/Craniofacial Association, American College of Surgeons, American Laryngological Rhinological and Otological Society, American Medical Association, Christian Medical & Dental Society, and Colorado Medical Society
Disclosure: Nothing to disclose.

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine
Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society
Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation unstricted gift unknown; Axis Three Corporation Ownership interest Consulting; Omni Biosciences Ownership interest Consulting; Sentegra Ownership interest Board membership; Syndicom Ownership interest Consulting; Oxlo  Consulting; Medvoy Ownership interest Management position

 
 
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