Ameloblastoma is an odontogenic neoplasm of enamel organ type tissue which does not undergo transformation to the point of enamel formation. We present the second case in the English literature of maxillary ameloblastoma that presented with nasal obstruction and rhinorrhoea, and the first to be excised using a combined maxillotomy and endoscopic ethmoidectomy. The patient had no previous dental history. The unusual presenting symptoms, as well as the highly destructive nature of these lesions when arising in the maxilla, make them worthy of consideration in the differential diagnosis of nasal and maxillary masses. We discuss the clinical features, pathology and management of these lesions and review the literature.

A case of melanotic neuroectodermal tumour of infancy is decribed. The pre-operative diagnosis was made on cytological material obtained by fine needle aspiration. The patient was a three-month-old male infant with a rapidly growing maxillary tumour mass that also involved the pterygomaxillary fossae and the floor of the orbit. In addition to the typical clinical presentation, the cytology is also distinctive showing a dual population of small neuroblastic cells and large melanin-containing epithelial cells. Histological, immunohistochemical and electron microscopic examination of the excised mass confirmed the initial diagnosis. The pre-operative distinction of this tumour from other small round cell tumours of infancy (rhabdomyosarcoma, neuroblastoma, melanoma and lymphoma), is essential in order to plan the most complete resection therefore reducing the possibilities of tumour recurrence. This tumour belongs to a field of pathology with which many otolaryngologists may not be familiar.

The melanotic neuroectodermal tumour of infancy is a rare, pigmented neoplasm that most frequently arises from the anterior maxillary alveolar ridge. It presents in the first few months of life, and usually follows a benign course. A classical case is presented and the clinical, radiological and pathological characteristics are discussed. The importance of early conservative surgical excision is emphasized with, in general, a good prognosis.

This tumour, which many otolaryngologists may not be familiar with, should be included in the differential diagnosis of head and neck neoplasms in infants and young children.

A case is described of ameloblastoma of maxilla presenting with numerous calcified keratin pearls. The significance of cellular variation in relation to the behavioural potential of the ameloblastoma in general is briefly discussed.