Brooke-Spiegler Syndrome with Multiple Scalp Cylindromas and Bilateral Parotid Gland Adenomas. (12th February 2012)

• Record Type: Journal Article
• Authors: Kalina, Peter; el-Azhary, Rokea
• Other Names: Bölke E. Academic Editor.; Corr P. D. Academic Editor.
• Is Part Of: Case reports in radiology. Volume 2012(2012)
• Publication Date: 2012-02-12
• Subjects: Radiology -- Periodicals; Radiology -- Case studies -- Periodicals; Medical radiology -- Periodicals; Radiology; Radiology; Medical radiology; Periodicals; Electronic journals; Case studies; Periodicals; 616.0757
• DOI: 10.1155/2012/249583
• Languages: English
• ISSNs: 2090-6862

Abstract

Abstract : A 62-year-old female presented with numerous soft tissue lesions of her scalp and bilateral preauricular region. Several of these have been biopsied or removed with a diagnosis of cylindromas. Cylindromas are benign tumors with a differentiation towards apocrine sweat glands that increase in number and size throughout life. Multiple scalp cylindromas may coalesce and cover the entire scalp, resulting in the "turban tumor." These are often associated with the autosomal dominant Brooke-Spiegler syndrome with coexistent facial trichoepitheliomas and spiradenomas. There is a very rare association between cylindromas and basal cell adenoma and adenocarcinoma of the parotid gland, with only 17 reported cases. Ours is the first CT demonstration of both the scalp and parotid gland findings in this uncommon situation.