Head and Neck Imaging

Angiofibroma

uncommon, highly vascular, locally invasive benign tumour which originates almost exclusively in the posterior nasal cavity of adolescent males. The tumour has the potential to kill or to cause serious morbidity due to uncontrolled growth, and sometimes also indirectly due to treatment complications. The prefix 'juvenile' is commonly used, but the lesion also occurs in childhood and (rarely) middle age. The tumour has been reported to occur in young females, but these examples are so exceptional that some authors state that sex chromosome studies are indicated if the diagnosis is confirmed in a female patient. The onset of symptoms occurs between 7 and 21 years of age. Symptoms include nasal obstruction, recurrent severe epistaxis, purulent rhinorrhoea, facial deformity and nasal speech. The diagnosis can usually be made on the basis of history, physical examination and imaging studies. The tumour appears clinically as a deep red mass with increased vascularity of the overlying mucosa. Biopsy is seldom required and dangerous to perform.

Imaging

On CT and MRI, the tumour appears as a strongly contrast-enhancing mass lesion in the posterior nasal cavity and nasopharynx. Intratumoral flow voids are present on MR images. The mass lesion is typically centred on the sphenopalatine foramen and very often extends into the pterygopalatine fossa, resulting in widening of this fossa with anterior bowing of the posterior maxillary wall (Fig.1). As the tumour grows, it tends to extend into the ipsilateral nasal cavity. Invasion of the neighbouring skull base structures is often seen; in more than half of patients the sphenoid sinus is invaded through the roof of the nasopharynx. Orbital and infratemporal spread are not uncommon. In large tumours, even intracranial extension may be seen. Angiography shows a hypervascular tumour, mainly supplied by the internal maxillary artery and ascending pharyngeal artery; this procedure is not usually needed for diagnosis, but is performed for presurgical tumour embolization. Natural regression almost never occurs. The tumour is radiosensitive, but the preferred therapy is complete surgical resection after selective tumour embolization. The prognosis is good, although fatalities due to haemorrhage are possible. Recurrences are probably related to incomplete resection, due to the multilobar nature of the tumour and its ability to invade adjacent spaces.

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Fig.1

MR images of a male adolescent presenting with recurrent epistaxis. The axial T2-weighted image (a) shows a mass lesion (arrows) centred on the left pterygopalatine fossa, anteriorly displacing the posterior maxillary wall and extending into the skull base; some flow voids are visible in the lesion. The axial T1-weighted image before (b) and after after injection of gadolinium (c) demonstrates the dramatic enhancement of the lesion. Normal right pterygopalatine fossa (arrowhead). Angiography (d) confirms the hypervascularity of the lesion, which is supplied by a hypertrophic maxillary artery (arrow).
Angiofibroma, Fig.1 (a)
Angiofibroma, Fig.1 (b)
Angiofibroma, Fig.1 (c)
Angiofibroma, Fig.1 (d)