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Skin - Nonmelanocytic tumors

Adnexal tumors - eccrine sweat glands

Eccrine cylindroma

Reviewer: Christopher Hale, M.D. (see Reviewers page)
Revised: 3 July 2012, last major update June 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● Also called turban tumor, particularly when a large, multicentric scalp tumor; dermal cylindroma
● Somewhat common
● Solitary, small, slow growing adenoma
● 90% in head and neck
● Usually ages 40+ years, 90% women
● Rarely associated with similar tumors in major salivary glands

Familial form (turban tumor syndrome, Brooke-Spiegler syndrome):
● Autosomal dominant, multiple tumors of children / teenagers that may also involve trunk and extremities, or in association with spiradenoma, trichoepithelioma, milia or membranous variant of basal cell adenoma of salivary glands
● Due to mutations in CYLD gene at chromosome 16q12-13
● Rarely undergoes malignant transformation (J Dermatol Case Rep 2007 Dec 29;1(1):4)

Spiradenocylindromas:
● Features of cylindroma and eccrine spiradenoma
● Usually painful

Clinical images
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Multiple scalp cylindromas forming turban tumor in patient with Brooke-Spiegler syndrome

Gross description
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● Pink-red dome-shaped nodule with smooth surface

Micro description
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● Compact nests of basaloid cells that fit together like a jigsaw puzzle, surrounded by thick basement membrane

Micro images
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Cylindroma

Molecular description
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● Mutation in CYLD gene in #16 associated with multiple cylindromas

Electron macroscopy description
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● Differentiation towards intradermal coiled duct region of eccrine sweat glands

Differential diagnosis
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● Eccrine spiradenoma

End of Skin - Nonmelanocytic tumors > Adnexal tumors - eccrine sweat glands > Eccrine cylindroma

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