Juvenile Angiofibroma

The juvenile angiofibroma is an uncommon tumor that primarily affects adolescent males. Although a histologically benign tumor, and one whose growth characteristics are often expansive rather than invasive, the destruction potential of juvenile angiofibromas should not be overlooked; their capacity for aggressive local growth remains an ongoing and significant source of morbidity. Most of these tumors arise extracranially, with the medial pterygoid region being their most common site of origin. With progressive growth, they involve the nose, nasopharynx, infratemporal fossa, sphenoid bone, cavernous sinus, inferior and superior orbital fissures, and middle cranial fossae. Because one of their routes of intracranial access involves superior extension through the roof of the sphenoid sinus into the sella, sellar and/or parasellar involvement is occasionally a feature of juvenile angiofibromas. Their most common presenting symptoms include recurrent nasal obstruction and epistaxis, the latter occasionally being quite dramatic. Additional symptoms include facial swelling, proptosis attendant to orbital extension, and cranial nerve palsies owing to optic nerve compression and cavernous sinus involvement. Evidence of pituitary dysfunction is unusual. In addition to demonstrating an enhancing soft tissue mass involving the nasopharynx and the skull base, the classic CT finding is widening of the pterygopalatine fossa. MRI clearly identifies the extent of both intracranial and extracranial disease and provides some indication of the vascularity of the tumor mass. Angiography is useful not only because of its classic appearance (early arterial blush with external carotid artery feeders) but also because of its therapeutic value, serving as a prelude to embolization.

Therapeutic options for angiofibromas, depending on their extent, include surgical resection, radiotherapy, and chemotherapy, either alone or in combination. Surgery and radiotherapy are each sufficiently effective as primary treatment modalities so that the relative role of each is controversial (115,116). Nevertheless, surgical resection remains the most widely used form of therapy, particularly in the case of smaller tumors, where complete surgical resection alone is often curative. Larger and incompletely removed tumors are often treated with adjuvant radiotherapy. Because these tumors arise extracranially and the bulk of the tumor resides there, most tumors are approached extracranially. Tumors with significant intracranial extension require combined craniofacial (extracranial-intracranial) approaches. Harrison, in a personal series of 44 patients, found that curative resections were achieved in 77% of patients. Recurrent tumors were often successfully treated by repeat resections (116).

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  • calimero conti
    Are the angiofibromas a tumor?
    8 years ago

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