Juvenile angiofibroma: evolution of management

Abstract

Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for juvenile angiofibroma. Refinement in preoperative embolization has provided significant reduction of complications and intraoperative bleeding with minimal risk of residual disease. During the last decade, an endoscopic technique has been extensively adopted as a valid alternative to external approaches in the management of small-intermediate size juvenile angiofibromas. Herein, we review the evolution in the management of juvenile angiofibroma with particular reference to recent advances in diagnosis and treatment.

Figure 1

Microscopic appearance of JA (hematoxylin-eosin staining (a) and immunohistochemistry for factor VIII (b)). Vessel caliber is extremely variable, the muscular layer of vessels is frequently absent, and stromal cells have usually a spindle-shaped appearance.

Figure 2

Axial contrast enhanced MRI: extensive JA with a typical pattern of spread into the cancellous bone of the basisphenoid along the vidian canal (white dotted line); on the contralateral side, black arrows indicate the right vidian nerve. Moreover, the lesion spreads deeply into the pterygomaxillary fossa toward the masticatory muscles, with anterior displacement of the posterior maxillary wall (white arrowheads). Asterisks indicate the foramen ovale bilaterally. TM: temporalis muscle; MM: masseter muscle.

Figure 3

Axial (a) and coronal (b) contrast-enhanced MRI. JA with its epicenter into the root of the left pterygoid process. The nasopharyngeal component with submucosal spread is clearly evident (black asterisk). The lesion reaches the intracranial extradural compartment through the inferior and superior orbital fissures (white arrows), inferolaterally displacing the maxillary nerve (black arrowhead). The white asterisk indicates Meckel's cave. LPM: lateral pterygoid muscle; MPM: medial pterygoid muscle.

Figure 4

Endoscopic appearance of JA showing a lobulated hypervascularized mass with a smooth surface partially covered by fibrin growing into the left nasal fossa. NS: nasal septum; IT: inferior turbinate; JA: juvenile angiofibroma.