Juvenile Nasopharyngeal Angiofibroma 

  • Author: Ted L Tewfik, MD, FRCSC; Chief Editor: Arlen D Meyers, MD, MBA   more...
 
Updated: Jul 26, 2011
 

Background

Juvenile angiofibroma (JNA) is a benign tumor that tends to bleed and occurs in the nasopharynx of prepubertal and adolescent males.

Next

History of the Procedure

Hippocrates described the tumor in the 5th century BC, but Friedberg first used the term angiofibroma in 1940. Other titles (eg, nasopharyngeal fibroma, bleeding fibroma of adolescence, fibroangioma) have also been used.

The image below depicts a coronal CT scan.

Coronal CT scan of the lesion filling the left nasCoronal 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.
Previous
Next

Epidemiology

Frequency

Juvenile nasopharyngeal angiofibroma (JNA) accounts for 0.05% of all head and neck tumors. A frequency of 1:5,000-1:60,000 in otolaryngology patients has been reported.

Sex

Juvenile nasopharyngeal angiofibroma (JNA) occurs exclusively in males. Females with juvenile nasopharyngeal angiofibroma (JNA) should undergo genetic testing.

Age

Onset is most commonly in the second decade; the range is 7-19 years. Juvenile nasopharyngeal angiofibroma (JNA) is rare in patients older than 25 years.

Previous
Next

Etiology

The lesion originates in close proximity to the posterior attachment of the middle turbinate, near the superior border of the sphenopalatine foramen.

A hormonal theory has been suggested because of the lesion's occurrence in adolescent males.

Other theories include a desmoplastic response of the nasopharyngeal periosteum or the embryonic fibrocartilage between the basiocciput and the basisphenoid.

Etiology from nonchromaffin paraganglionic cells of the terminal branches of the maxillary artery has also been suggested. Comparative genomic hybridization analysis of these tumors revealed deletions of chromosome 17, including regions for the tumor suppressor gene p53 as well as the Her-2/neu oncogene.

Previous
Next

Pathophysiology

The tumor starts adjacent to the sphenopalatine foramen. Large tumors are frequently bilobed or dumbbell-shaped, with one portion of the tumor filling the nasopharynx and the other portion extending to the pterygopalatine fossa.

Anterior growth occurs under the nasopharyngeal mucous membrane, displacing it anteriorly and inferiorly toward the postnasal space. Eventually, the nasal cavity is filled on one side, and the septum deviates to the other side. Superior growth is directed toward the sphenoid sinus, which may also be eroded. The cavernous sinus may become invaded if the tumor advances further.

Lateral spread is directed toward the pterygopalatine fossa, bowing the posterior wall of the maxillary sinus. Later, the infratemporal fossa is invaded. Occasionally, the greater wing of the sphenoid may be eroded, exposing the middle fossa dura. Proptosis and optic nerve atrophy result if orbital fissures are encroached upon by the tumor. Extranasopharyngeal angiofibroma is extremely rare and tends to occur in older patients, predominately in females, but the tumor is less vascular and less aggressive than juvenile nasopharyngeal angiofibroma (JNA).

Previous
Next

Presentation

Symptoms

  • Nasal obstruction (80-90%) - Most frequent symptom, especially in initial stages
  • Epistaxis (45-60%) - Mostly unilateral and recurrent; usually severe epistaxis that necessitates medical attention; diagnosis of angiofibroma in adolescent males to be ruled out
  • Headache (25%) - Especially if paranasal sinuses are blocked
  • Facial swelling (10-18%)
  • Other symptoms - Unilateral rhinorrhea, anosmia, hyposmia, rhinolalia, deafness, otalgia, swelling of the palate, deformity of the cheek

Signs

  • Nasal mass (80%)
  • Orbital mass (15%)
  • Proptosis (10-15%)
  • Other signs include serous otitis due to eustachian tube blockage, zygomatic swelling, and trismus that denote spread of the tumor to the infratemporal fossa, decreasing vision due to optic nerve tenting (rare)

Differentials

Previous
Proceed to Workup
 
 
Contributor Information and Disclosures
Author

Ted L Tewfik, MD, FRCSC  Professor, Department of Otolaryngology-Head and Neck Surgery, Director of Continuing Medical Education of Otolaryngology, McGill University Medical School; Director of Professional Affairs of Otolaryngology, Division of Otolaryngology, Montreal Children's Hospital; Senior Staff, Montreal General Hospital and Royal Victoria Hospital

Ted L Tewfik, MD, FRCSC, 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  Consultant, Department of Otolaryngology Head and Neck Surgery, King Abdulaziz Medical City, Jeddah, Saudi Arabia

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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.

Christopher L Slack, MD  Private Practice in Otolaryngology and Facial Plastic Surgery, 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 Unrestricted 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; Cerescan Imaging Honoraria Consulting; GYRUS ACMI Honoraria Consulting

References

  1. Wu AW, Mowry SE, Vinuela F, Abemayor E, Wang MB. Bilateral vascular supply in juvenile nasopharyngeal angiofibromas. Laryngoscope. Mar 2011;121(3):639-43. [Medline].

  2. 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].

  3. 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].

  4. Chakraborty S, Ghoshal S, Patil VM, Oinam AS, Sharma SC. Conformal radiotherapy in the treatment of advanced juvenile nasopharyngeal angiofibroma with intracranial extension: an institutional experience. Int J Radiat Oncol Biol Phys. Aug 1 2011;80(5):1398-404. [Medline].

  5. 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].

  6. Mattei TA, Nogueira GF, Ramina R. Juvenile Nasopharyngeal Angiofibroma with Intracranial Extension. Otolaryngol Head Neck Surg. May 13 2011;[Medline].

  7. Aziz-Sultan MA, Moftakhar R, Wolfe SQ, Elhammady MS, Herman B, Farhat H. Endoscopically assisted intratumoral embolization of juvenile nasopharyngeal angiofibroma using Onyx. J Neurosurg Pediatr. Jun 2011;7(6):600-3. [Medline].

  8. 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].

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

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

  11. 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].

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

  13. 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].

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

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

  16. 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.

  17. 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].

  18. 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].

  19. 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].

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

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

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

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

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

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

 
Previous
Next
 
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 cavity and paranasal sinuses. Courtesy of J Otolaryngol 1999;28:145.
Coronal MRI scan showing extension of the lesion to the cavernous sinus. Courtesy of J Otolaryngol 1999;28:145.
Angiogram depicting angiofibroma before embolization. Courtesy of J Otolaryngol 1999;28:145.
Angiogram depicting angiofibroma after embolization. Courtesy of J Otolaryngol 1999;28:145.
Preembolization lateral carotid angiogram of juvenile nasopharyngeal angiofibroma (JNA).
Postembolization angiogram.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.