You are in: eMedicine Specialties > Vascular Surgery > MEDICAL TOPICS AngiofibromaArticle Last Updated: Jun 26, 2006AUTHOR AND EDITOR INFORMATIONSection 1 of 12
  Author: Eric Mansfield, MD, Consulting Staff, Department of Otolaryngology, Cape Fear Otolaryngology Eric Mansfield is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery and National Medical Association Coauthor(s): Frederick Shuler, MD, Consulting Vascular Surgeon, Department of Surgery, Vascular Associates, LLC; Ira Uretsky, MD, Assistant Chief, Department of Otolaryngology, Womack Army Medical Center; David Moody, MD, Consulting Staff, Department of General and Interventional Radiology, North Idaho Imaging Center; Edward Dickerson, MD, Chief of Otolaryngology, Head and Neck Surgery, Department of Surgery, Womack Army Medical Center; Assistant Professor, Department of Otolaryngology, Cape Fear Otolaryngology Editors: Jeffrey Lawrence Kaufman, MD, Associate Professor, Department of Surgery, Division of Vascular Surgery, Tufts University School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Travis J Phifer, MD, Chief, Division of Vascular Surgery, Professor, Department of Surgery and Radiology, Louisiana State University Health Sciences Center in Shreveport; Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy; William H Pearce, MD, Chief, Division of Vascular Surgery, Violet and Charles Baldwin Professor of Vascular Surgery, Department of Surgery, Northwestern University School of Medicine Author and Editor Disclosure Synonyms and related keywords: angiofibroma, juvenile angiofibroma, juvenile nasopharyngeal angiofibroma, JNA, nasal cavity tumor, nasal tumor, benign nasal tumor, nose tumor, nasopharyngeal tumor INTRODUCTIONSection 2 of 12
  Juvenile nasopharyngeal angiofibroma (JNA) is one of the most common benign nasal cavity tumors of adolescence. It often acts in a malignant manner by eroding into the surrounding sinuses, orbit, or cranial vault. History of the ProcedureAlthough Hippocrates described lesions similar to JNAs, Chelius associated the tumor with adolescent males in 1847. A review by Martin et al in 1948 defined most of the associated features described below. The first successful surgical resection of a probable JNA is credited to Liston in 1841 at University College Hospital in London. FrequencyIt is found almost exclusively in adolescent males (average age, 14-18 y). Young females given this diagnosis should undergo chromosomal studies or should have the diagnosis questioned. Incidence of JNA is 1 case per 5000-60,000 ear, nose, and throat patients and accounts for 0.5% of all head and neck tumors. The wide range of reported cases may stem from misdiagnosis and inclusion of other lesions. Incidence is reported to be higher in Egypt and India. EtiologyThe cause has not been determined. The most accepted theory is that JNAs originate from sex steroid–stimulated hamartomatous tissue located in the turbinate cartilage. The proposed hormonal influence may explain why (rarely) some JNAs involute after puberty. Recent studies by Bretani demonstrate estrogen and progesterone receptors in JNA, but gonadotropin levels in all patients are normal. Another proposed theory includes tumor originating from embryonal chondrocartilage of the occipital plate. PathophysiologyThe proposed origin of the JNA is located along the posterior-lateral wall in the roof of the nasopharynx, usually in the region of the superior margin of the sphenopalatine foramen and the posterior aspect of the middle turbinate. Fetal histology confirms large areas of endothelial tissue in this region. Rather than invading surrounding tissue, this tumor displaces and distorts, relying on pressure necrosis to destroy and push through its bony confines. Intracranial extension is noted in 10-20% of cases. ClinicalSigns and symptoms are present for an average of 6 months prior to the diagnosis, commonly with extension beyond the nasopharynx. Symptoms
Signs
Differential diagnosis
See Staging. INDICATIONSSection 3 of 12
Symptoms of invasion to surrounding tissues, chronic nosebleeds, and nasal obstruction portend the need for prompt surgical intervention. Enlargement of a known JNA also may indicate the need for surgical intervention. RELEVANT ANATOMYSection 4 of 12
From its origin along the posterior middle turbinate, the JNA extends inferiorly, displacing the soft palate, eroding into the hard palate, and medially displacing the nasal septum. Filling the nasal cavity, it extends anteriorly through the posterior wall of the maxillary sinus, laterally through the pterygomaxillary fissure into the temporal and infratemporal fossa, and superiorly into the orbit and cranial cavity. JNAs most commonly are fed by the internal maxillary artery; however, with growth of the tumor, surrounding vessels may be parasitized. CONTRAINDICATIONSSection 5 of 12
Surgical contraindications include extension into or involvement of unresectable intracranial contents. Involvement of the lateral wall of the cavernous sinus is considered to be resectable in specific cases. WORKUPSection 6 of 12
Lab Studies
Imaging Studies
Diagnostic Procedures
Histologic FindingsFindings include mature fibrous tissue containing variable amounts of thin-walled vessels. These vessels are lined with endothelium, but they lack the normal contractile muscular elements in their vessel walls. This may explain their propensity to bleed. StagingUsed for prognosis and therapeutic approaches, it was proposed by multiple surgeons. It is based on CT scan findings.
TREATMENTSection 7 of 12
Medical therapyExternal beam irradiation: This most often is reserved for intracranial, unresectable, or recurrent disease. Variable dosing schemes of 30-46 Gy are used. It rapidly resolves symptoms, but tumor resolution can take months to years. Residual tumor present 2 years after treatment often recurs. Major concerns include secondary skin, bone, soft tissue, and thyroid malignancies and of inhibition of facial bone growth. Chemotherapy: Hormonal flutamide, a nonsteroidal androgen blocker, interferes with testosterone binding and has been shown to reduce tumor size. Hormonal therapy with diethylstilbestrol (5 mg PO tid for 6 wk) before excision has been shown to diminish the vascularity of the JNA but is associated with feminizing side effects. Neither is used routinely; their use is isolated to clinical trials. Doxorubicin and dacarbazine are reserved for recurrences. Cryotherapy has been used in the past but no longer is a mainstay. Surgical therapyThis remains the mainstay of therapy for stage I-IVa tumors. Several approaches are dependent on the location and extent of the JNA. Options include any combination of lateral rhinotomy and lip split; midface degloving; or transpalatal, transantral, or transzygomatic infratemporal approach. Preoperative detailsPlanning of the approach is based on CT scan, MRI, and angiography findings. Preoperative angiography provides embolization of the tumor and reduces operative blood loss by 66% if performed within 48 hours of surgery. Follow-upRecurrences are treated with radiation or, occasionally, reoperation. COMPLICATIONSSection 8 of 12
Complications inherently are related to intracranial extension (stage IV disease), uncontrolled hemorrhage and death, iatrogenic injury to vital structures, and perioperative transfusions. OUTCOME AND PROGNOSISSection 9 of 12
Preoperative embolization decreases morbidity and recurrence. Cure rates for primary surgery are near 100% with complete resection of extracranial JNAs and 70% with intracranial tumors. A 90% cure rate is associated with second surgery for recurrences. FUTURE AND CONTROVERSIESSection 10 of 12
The presence of angiogenic protein basic growth factor may mediate the proliferation of JNA, and its modulation may lead to a better noninvasive treatment modality. Most feel that spontaneous regression without treatment does not occur, despite possible hormonal influence. Recent occurrence of 6 cases of JNA in patients with Gardner syndrome may lead to consideration of a genetic influence. MULTIMEDIASection 11 of 12
REFERENCESSection 12 of 12
Article Last Updated: Jun 26, 2006 | |||||||||||||||||||
