Juvenile Angiofibroma

juvenile angiofibroma Gd axial

This 15 year-old male patient presented with nasal obstruction. The gadolinium-enhanced T1-weighted fat-saturated MR image above shows an enhancing mass in the right nasal cavity which appears centred on the sphenopalatine foramen and extends into the pterygopalatine fossa. T2 fat-sat axial image shows flow voids within the lesion, consistent with high-flow vascularity. Coronal MRA shows an enlarged maxillary artery feeding the tumour.

Juvenile angiofibroma is a locally aggressive benign tumour found almost exclusively in adolescent males. The origin of the tumour is unclear but may be due to hormonal influence on a hamartomatous rest in the nasopharynx. Tumours typically arise from the posterior middle turbinate or superior margin of the sphenopalatine foramen. The tumour does not invade but displaces and distorts surrounding tissue and bone. Ten to twenty percent show intracranial extension. Presentation may be with epistaxis, nasal obstruction, eustachian tube obstruction, diplopia, or anosmia. Advanced cases may show a cheek mass, bulging palate or proptosis.

Plain film findings include anterior bowing of the posterior wall of the maxillary antrum, widening of the pterygomaxillary and superior orbital fissures, or nasal septal deviation. CT or MRI will show the extent of soft tissue tumour. Angiography will demonstrate feeding vessels, which may be bilateral, and may arise from distant arteries such as vertebral or thyrocervical (although the usual feeder is the maxillary artery).

Biopsy of these lesions is not recommended due to high tumour vascularity.

The mainstay of treatment is surgical excision. Preoperative embolisation has been shown to significantly reduce intraoperative blood loss. Radiotherapy is usually reserved for unresectable (involving the cavernous sinus, pituitary or optic chiasm) or recurrent tumour. Hormonal treatment is experimental at present.

The prognosis is excellent for tumours without intracranial extension, and good for those with.

References:
1. eMedicine, June 2006
2. Fordice, JO. Juvenile Nasopharyngeal Angiofibroma, Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, 1993

Credit: Dr Laughlin Dawes