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Turban tumour with involvement of the parotid gland

Published online by Cambridge University Press:  29 June 2007

Markus Jungehülsing*
Affiliation:
Departments of Otorhinolaryngology and Pathology* of the University of Cologne Medical School, Cologne, Germany.
Matthias Wagner
Affiliation:
Departments of Otorhinolaryngology and Pathology* of the University of Cologne Medical School, Cologne, Germany.
Michael Damm
Affiliation:
Departments of Otorhinolaryngology and Pathology* of the University of Cologne Medical School, Cologne, Germany.
*
Address for correspondence: Markus Jungehülsing, M.D., Universitäts-HNO-Klinik Köln, Joseph-Stelzmann-Str. 9, 50924 Köln, Germany. Fax: 49 221 478 4793 e-mail: [email protected]

Abstract

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.

Type
Pathology in Focus
Copyright
Copyright © JLO (1984) Limited 1999

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