Juvenile Angiofibroma

Fig. 2.8. Juvenile angiofibroma. a Polypoid mass of white-red cut surface and rubbery consistency. b Vascular elements embedded in fibrous tissue showing intravascular microembolisation, a treatment modality prior to surgery entiate neurofibromas from schwannomas of the sinona-sal mucosa. Neurofibromas should also be distinguished from myxomas, which are S-100 protein-negative.


Meningiomas of the sinonasal tract may extend directly from the central nervous system or arise from ectopic extracranial tissue. Although always rare, they are more commonly seen in the orbit, ear and skin of the head and neck than in the sinonasal tract. Histologically, they are similar to meningiomas elsewhere, the meningothelial type being the most frequent. Sinonasal meningiomas tend to occur in younger patients than intracranial me-ningiomas [118, 203].


There are few reports on nasal paragangliomas. The tumours originate in the middle turbinates and ethmoid


Juvenile nasopharyngeal angiofibroma arises in the confluence of the posterolateral nasal wall and the lateral nasopharynx and occurs almost exclusively in males during adolescence [90, 125]. The tumour is sessile or polypoid (Fig. 2.8a) and is histologically benign, but has a tendency to recur and is locally destructive, causing pressure necrosis of adjacent soft tissue and bone. It may occasionally extend into paranasal sinuses, orbit and cranial fossae. It is composed of vascular and fibrous elements in varying proportions. The vessels in the superficial portions of the tumour are mainly gaping capillaries that may become compressed with increasing stromal fibrosis. Thick-walled vessels without elastic membranes and with irregular, incomplete or absent muscle coats and focal intimal thickenings are usually present in the deeper portions of the tumour. These vessels resemble those normally seen in the sub-mucosa of the nasal conchae. The vascular elements are embedded in fibrous tissue, which varies in cellularity and collagenisation. Stellate fibroblast-like cells are often present close to the blood vessels. The fibroblastic cells of nasopharyngeal angiofibroma are strongly positive for testosterone receptors [120]. Ultrastructur-ally, the nuclei of angiofibroma contain characteristic dense granules [251]. Occasionally, the fibroblasts may exhibit cytologic atypia, and some of these cells may be multinucleated, but mitosis is rare. Mast cells may be numerous. There may be focal thrombosis, haemorrhage and chronic inflammatory reaction. With the advent of preoperative selective embolisation, iatrogenic emboli (Fig. 2.8b) are increasingly encountered in resected specimens [232]. For more details on this tumour see Chap. 6.

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