eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Pediatric Otolaryngology

Juvenile Nasopharyngeal Angiofibroma

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
Mohammed A Al Garni, MBBS, Fellow, Department of Otolaryngology, McGill University, Canada

Updated: Aug 27, 2009

Introduction

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

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.

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.

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.

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.

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.

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

• Other causes of nasal obstruction, (eg, nasal polyps, antrochoanal polyp, teratoma, encephalocele, dermoids, inverting papilloma, rhabdomyosarcoma, squamous cell carcinoma)
• Other causes of epistaxis, systemic or local
• Other causes of proptosis or orbital swellings

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 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.

• 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 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 embolization. Courtesy of J Otolaryngol 1999;28:145.

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

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.

Outcome and Prognosis

The presence of tumor in the pterygoid fossa and basisphenoid, erosion of the clivus, intracranial extension, feeders from the internal carotid artery, a young age, and a residual tumour were risk factors associated with the recurrence of juvenile nasopharyngeal angiofibroma.

Multimedia

Media file 1: 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.

Media file 2: Axial CT scan of lesion involving the right nasal cavity and paranasal sinuses. Courtesy of J Otolaryngol 1999;28:145.

Media file 3: Coronal MRI scan showing extension of the lesion to the cavernous sinus. Courtesy of J Otolaryngol 1999;28:145.

Media file 4: Angiogram depicting angiofibroma before embolization. Courtesy of J Otolaryngol 1999;28:145.

Media file 5: Angiogram depicting angiofibroma after embolization. Courtesy of J Otolaryngol 1999;28:145.

Media file 6: Preembolization lateral carotid angiogram of juvenile nasopharyngeal angiofibroma (JNA).

Media file 7: Postembolization angiogram of same patient in Image 6.

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].

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

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
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