eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Pediatric Otolaryngology

Juvenile Nasopharyngeal Angiofibroma: Treatment

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

Treatment

Medical Therapy

  • Hormonal therapy
    • The testosterone receptor blocker flutamide was reported to reduce stage I and II tumors to 44%.
    • Despite tumor reduction with hormones, this approach is not routinely used.
    • Schuon et al reported on the immunohistochemical analysis of growth mechanisms in juvenile nasopharyngeal angiofibroma.1 They concluded that juvenile angiofibroma (JNA) growth and vascularization are driven by factors released from stromal fibroblasts. Therefore, inhibition of these factors might be beneficial for the therapy of inoperable juvenile nasopharyngeal angiofibroma (JNA).
    • Radiotherapy
      • Some centers have reported 80% cure rates with radiation therapy. However, concerns regarding potential effects of radiation make radiation therapy a nonuseful modality in most cases.
      • Stereotactic radiotherapy (ie, Gamma Knife) delivers a lower dose of radiation to surrounding tissues. However, most authorities reserve radiotherapy for intracranial disease or recurrent cases.
      • Three-dimensional conformal radiotherapy in extensive juvenile nasopharyngeal angiofibroma (JNA) or intracranial extension provides a good alternative to conventional radiotherapy regarding disease control and radiation morbidity.

Surgical Therapy

  • A lateral rhinotomy, transpalatal, transmaxillary, or sphenoethmoidal route is used for small tumors (Fisch stage I or II).
  • The infratemporal fossa approach is used when the tumor has a large lateral extension.
  • The midfacial degloving approach, with or without a LeFort osteotomy, improves posterior access to the tumor.
  • The facial translocation approach is combined with Weber-Ferguson incision and coronal extension for a frontotemporal craniotomy with midface osteotomies for access.
  • An extended anterior subcranial approach facilitates en bloc tumor removal, optic nerve decompression, and exposure of the cavernous sinus.
  • Intranasal endoscopic surgery: Some authors advocate its use for lesions with limited extension to the infratemporal fossa. Image-guided, endoscopic, laser-assisted removal has also recently been used. Hackman et al (2009) reviewed 31 cases of JNA at the University of Pittsburgh Medical Center from 1995 to 2006.2 Most tumors were completely excised using the expanded endonasal approach (EEA) alone or in combination with minor sublabial incisions, avoiding the morbidity associated with larger open approaches or postoperative radiation therapy.

Preoperative Details

Preoperative embolization can be accomplished using reabsorbable microparticulate substances (eg, Gelfoam, polyvinyl alcohol, dextran microspheres) or nonabsorbable microparticulates (eg, Ivalon, Terbal). Limit blood loss during surgery is essential.

Complications

Preoperative angiography and embolization minimize intraoperative blood loss, and the current shift in the treatment to endoscopic excision in selected cases reduces perioperative morbidity.3 Low-grade consumption coagulopathy may complicate small juvenile nasopharyngeal angiofibroma (JNA) and implies that preoperative coagulation screening may have a role in perioperative hemostasis.

Malignant transformation has been reported in 6 cases; 5 of these patients were treated with radiotherapy, according to a study by Makek et al.4

Transient blindness has been reported as a result of embolization, but it is a rare occurrence. Osteoradionecrosis and/or blindness due to optic nerve damage may occur with radiotherapy.

Fistula of the palate at the junction of the soft and hard palate may occur with the transpalatal approach but is prevented by preservation of the greater palatine vessels during flap elevation.

Anesthesia of the cheek is a frequent occurrence with the Weber-Ferguson incision.

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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