Turban tumour with involvement of the parotid gland

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 t hird decade. With female preponderance dermal cylindromas predominantly ari se in hairy areas of the body with approximately 90 per cent on the head an d neck. Transformation to malignancy seems to be scarce. Although cylindrom as of the skin resemble basal cell adenomas of the salivary gland, there is usually no salivary gland involvement. On the other hand, patients with ba sal cell adenomas of a salivary gland usually do not show dermal lesions. W e report one of the rare cases of FADC combined with multiple basal cell ad enomas of the parotid glands and present a review of the literature.