Juvenile Nasopharyngeal Angiofibroma Workup

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

Imaging Studies

Plain radiography views 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 scan images below demonstrate the extent of the tumor. Extension to the sphenoid sinus, erosion of the greater sphenoidal wing, or invasion of the pterygomaxillary and infratemporal fossae is usually visualized, as in the images below.

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. 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, especially in cases of intracranial involvement. Coronal MRI scan showing extension of the lesion to the cavernous sinus is seen in the image below.

Coronal MRI scan showing extension of the lesion tCoronal 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. Bilateral vascular supply may be an underappreciated factor in JNA, and thorough radiographic investigation via angiography of bilateral carotid systems should be routinely performed preoperatively.[1] An angiofibroma before and after embolization can be seen in the images below.

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

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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
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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

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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.
 
 
 
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