The majority of tumours present late with involvement of important adjacent structures. Even histologically 'benign' tumours may be associated with significant morbidity and/or mortality at this site. The principle of oncological resection may be compromised by the proximity of vital structures. This, combined with the histo-logical diversity in this area, necessitates specialist management.
Diagnosis and assessment of extent of disease relies upon expert imaging and histology. Ideally, CT scanning (direct coronal and axial cuts with intravenous contrast enhancement) combined with MRI should be undertaken. In addition, a chest X-ray and CT of the thorax, abdomen, and bone studies may be appropriate if occult primary or systemic metastases are suspected.
Biopsy should ideally be performed under general anaesthesia to obtain representative tissue. Tissue should also be removed via an endonasal endoscopic approach. Surgery alone, or in combination with radiotherapy, is required in the majority of cases. Surgical options include craniofacial resection, maxillectomy, or occasionally total rhinectomy, with or without orbital exenteration. Prosthetic rehabilitation should be undertaken immediately when the palate is sacrificed.
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