Nasopharyngeal angiofibroma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Juvenile nasopharyngeal angiofibroma; angiofibroma of the nasopharynx; JNA

Overview

Nasopharyngeal angiofibroma (also called juvenile nasopharyngeal angiofibroma) is a locally aggressive, benign vascular neoplasm that grows in the pterygopalatine fossa. The most common symptoms of nasopharyngeal angiofibroma include one-sided nasal obstruction and recurrent bleeding. Nasopharyngeal angiofibroma may be classified according to Radkowski Classification System (severity) into 3 categories: I, II, and III. The majority of nasopharyngeal angiofibromas are irrigated by the external carotid artery. Genetic alterations associated with the development of nasopharyngeal angiofibroma include: overexpression of PDGF-B, bFGF, VEGF. Nasopharyngeal angiofibroma accounts for 0.05% of all head and neck tumors. Nasopharyngeal angiofibromas are more commonly observed among children and adolescents. Males are more commonly affected with nasopharyngeal angiofibroma than females. Common risk factors in the development of nasopharyngeal angiofibroma, include: presence of tumor in the pterygoid fossa and young age. Physical examination may be remarkable for smooth submucosal mass in the posterior nasal cavity. Computed tomography is the imaging modality of choice. On conventional radiography, findings of nasopharyngeal angiofibroma include: visualization of a nasopharyngeal mass, opacification of the sphenoid sinus, and anterior bowing of the posterior wall of the maxillary antrum. Surgery is the mainstay of therapy for nasopharyngeal angiofibroma. Surgical approach for nasopharyngeal angiofibroma will usually depend on the stage. The treatment of choice for early stage for nasopharyngeal angiofibroma is intranasal endoscopic surgery ( e.g. lateral rhinotomy). The treatment of choice for late stage nasopharyngeal angiofibroma include the infratemporal fossa approach, and the mid-facial degloving approach. Common complications of nasopharyngeal angiofibroma include transient blindness, optic nerve damage, and low-grade consumption coagulopathy.

Historical Perspective

• Nasopharyngeal angiofibroma was first described by Hippocrates, a Greek physician, in the 5th century BC.

Classification

• Nasopharyngeal angiofibroma may be classified according to Radkowski Classification System into 3 categories:
• Stage I

• Ia: limited to nasal cavity/nasopharynx
• Ib: extension into one or more paranasal sinuses

• Stage II

• IIa: minimal extension through sphenopalatine foramen into pterygomaxillary fossa
• IIb: fills pterygomaxillary fossa bowing the posterior wall of the maxillary antrum anteriorly or extending into the orbit via the inferior orbital fissure
• IIc: extends beyond pterygomaxillary fossa into infratemporal fossa

• Stage III

• Stage IIIA: intracranial extension

Pathophysiology

• The pathogenesis of nasopharyngeal angiofibroma is characterized by the following features:

• Vascular neoplasm
• Originates from the pterygopalatine fossa
• The majority are associated with the external carotid artery

• Genetic alterations associated with the development of nasopharyngeal angiofibroma include:

• Overexpression PDGF-B
• Overexpression bFGF
• Overexpression bFGF
• Deletion of chromosome 17
• Tumor suppressor gene p53
• Overexpression of Her-2/neu oncogene

• On gross pathology, characteristic findings of nasopharyngeal angiofibroma include:

• Unencapsulated
• Polypoid fibrous mass
• Bleeding on manipulation

• On microscopic histopathological analysis, characteristic findings of nasopharyngeal angiofibroma include:

• Fibroblastic cells with plump (near cuboidal) nuclei
• Fibrous stroma
• Abundant capillaries

Causes

• There are no known causes of nasopharyngeal angiofibroma.

Differentiating Nasopharyngeal Angiofibroma from Other Diseases

• Nasopharyngeal angiofibroma must be differentiated from other diseases that cause epistaxis, unilateral nasal obstruction, and rhinorrhea, such as:

• Antro-choanal polyp
• Rhinosporidiosis
• Chordoma
• Nasopharanageal cyst
• Pyogenic granuloma

Epidemiology and Demographics

• Nasopharyngeal angiofibroma is a rare tumor.
• Nasopharyngeal angiofibroma accounts for 0.05% of all head and neck tumors.
• The prevalence of nasopharyngeal angiofibroma is approximately 0.4 per 100,000 individuals worldwide.

Age

• Nasopharyngeal angiofibroma is more commonly observed among patients aged 7-19 years.

Gender

• The male to female ratio for nasopharyngeal angiofibroma is approximately 4 to 1.

Race

• There is no racial predilection for nasopharyngeal angiofibroma.

Risk Factors

• Common risk factors in the development of nasopharyngeal angiofibroma include:

• Presence of tumor in the pterygoid fossa
• Young age
• Feeders from the internal carotid artery
• Residual tumor

Natural History, Complications and Prognosis

• The majority of patients with nasopharyngeal angiofibroma are symptomatic at diagnosis.
• Early clinical features include epistaxis, facial pain, and headache.
• If left untreated, the majority of patients with nasopharyngeal angiofibroma may progress to develop malignant transformation.
• Common complications of nasopharyngeal angiofibroma include transient blindness, optic nerve damage, and low-grade consumption coagulopathy.
• Prognosis is generally good, and the 5-year survival rate of patients with early stage nasopharyngeal angiofibroma is approximately 90%
• Survival rate of patients with late stage nasopharyngeal angiofibroma is approximately 50% to 75%.

Diagnosis

Diagnostic Criteria

• The diagnosis of nasopharyngeal angiofibroma is made when the following diagnostic criteria are met:

• Clinical criteria

• Young patient
• Epistaxis

• Positive physical exam

• A smooth submucosal mass in the posterior nasal cavity

• Positive imaging finding: visualization of a nasopharyngeal mass

Symptoms

• Common symptoms of nasopharyngeal angiofibroma, may include:

• Epistaxis or blood-tinged nasal discharge
• Unilateral nasal obstruction
• Rhinorrhea
• Hearing loss
• Diplopia
• Anosmia (rare)
• Eye pain

Physical Examination

• Patients with nasopharyngeal angiofibroma usually are well-appearing.
• Physical examination may be remarkable for:

• A smooth submucosal mass in the posterior nasal cavity

Laboratory Findings

• There are no specific laboratory findings associated with the diagnosis of nasopharyngeal angiofibroma.

Imaging Findings

• Computed tomography is the imaging modality of choice for nasopharyngeal angiofibroma.
• On conventional radiography, findings of nasopharyngeal angiofibroma include:

• Visualization of a nasopharyngeal mass
• Opacification of the sphenoid sinus
• Anterior bowing of the posterior wall of the maxillary antrum
• Holman-miller sign: the anterior bowing of the posterior wall of the maxillary antrum which is seen on lateral skull film or cross-sectional imaging
• Widening of the pterygomaxillary fissure and pterygopalatine fossa
• Erosion of the medial pterygoid plate

• On CT, findings of nasopharyngeal angiofibroma include:

• Bony changes
• Non-encapsulated soft tissue mass
• Anterior bowing the posterior wall of the maxillary antrum

• On MRI, findings of nasopharyngeal angiofibroma include:

• T1: intermediate signal
• T2: heterogeneous signal - flow voids appear dark
• T1 C+ (Gd): shows prominent enhancement

• The images below demonstrate findings of nasopharyngeal angiofibroma.

• 
  Nasopharyngeal angiofibroma. Skull base invasionImage courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology
• 
  MRI (T1): Nasopharyngeal angiofibroma 1/3 Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology
• 
  MRI (T1): Nasopharyngeal angiofibroma Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology
• 
  MRI (T1): Nasopharyngeal angiofibroma 3/3 Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology
• 
  MRI (T1): Nasopharyngeal angiofibroma 3/3 (circled)Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

Other Diagnostic Studies

• Nasopharyngeal angiofibroma may also be diagnosed using nasal endoscopy.

Treatment

Medical Therapy

• Medical therapy for nasopharyngeal angiofibroma is divided into 2 categories:

• Hormonal therapy
• Radiotherapy

• Hormonal therapy for nasopharyngeal angiofibroma includes:

• Flutamide

• Medical treatment is usually given before surgery to reduce the blood loss
• Radiotherapy for nasopharyngeal angiofibroma, include:

• Stereotactic radiotherapy

Surgery

• Surgery is the mainstay of therapy for nasopharyngeal angiofibroma.
• Preoperative embolization is highly suggested for nasopharyngeal angiofibroma.
• Surgical approach for nasopharyngeal angiofibroma will depend on the stage.

• The treatment of choice for early stage for nasopharyngeal angiofibroma is intranasal endoscopic surgery. Lateral rhinotomy is the preferred surgical approach.
• The treatment of choice for late stage for nasopharyngeal angiofibroma include the infratemporal fossa approach, and the mid-facial degloving approach.

Prevention

• There are no primary preventive measures available for nasopharyngeal angiofibroma.
• Once diagnosed and successfully treated, patients with nasopharyngeal angiofibroma are followed-up after surgery, and every 6 or 12 months, depending on the clinical progression.