Familial autosomal dominant cylindromatosis (FADC, turban tumour syndrome, Brooke-Spiegler-syndrome and many more, MacKusick catalogue numbers 123850, 313100) is a rare hereditary disease usually presenting in the second or third decade. With female preponderance dermal cylindromas predominantly arise in hairy areas of the body with approximately 90 per cent on the head and neck. Transformation to malignancy seems to be scarce. Although cylindromas of the skin resemble basal cell adenomas of the salivary gland, there is usually no salivary gland involvement. On the other hand, patients with basal cell adenomas of a salivary gland usually do not show dermal lesions. We report one of the rare cases of FADC combined with multiple basal cell adenomas of the parotid glands and present a review of the literature.

A very rare case of a sebaceous carcinoma of the external auditory canal with basal cell differentiation is presented. Fewer than 400 cases affecting any part of the body have so far been reported and of that only seven cases have been known to involve the external auditory canal. The clinical features, pathology and treatment are described and the relevant literature has been reviewed.

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.