Case Number : Case 574 - 21 Aug Posted By: Guest

Eccrine spiradenoma.

The tumor is made of islands with jigsaw puzzle appearance and are surrounded with esinophilic hyaline membranes. I will diagnose it cylindroma.

It is a nice cylindroma

It´s more consistent with cylindroma

Cylindroma. Given that there are multiple lesions, could this be Brooke-Spiegler syndrome?

Cylindroma and Brooke-Spiegler

Cylindroma / Trichoepithelioma (? Brooke-Speigler sy)

cylindroma

[color=black][b]Spiegler's tumour[/b]. [b]Cylindroma [/b][/color][color="#000000"][b][Spiegler’s tumour] [/b][color=black] is a tumour of basaloid cells in the dermis arranged in islands that often fit together like a jigsaw puzzle ([b]jigsaw-puzzle architecture[/b]), the islands are surrounded by[/color] c[b]onspicuous eosinophilic band (hyaline[/b])[color=black] - apocrine/sweat duct [b]lu[b]mina[/b] [/b]often present within tumour islands - cylindromas are more likely to be related to the [b]apocrine[/b] gland than to the eccrine gland.[/color][/color]

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[color=black]Cylindromas are sometimes associated with multiple trichoepitheliomas [/color][color="#000000"][Brooke’s tumour][color=black] in the [b]Brooke–Spiegler syndrome [/b](with occasionally spiradenomas in addition) and also in [b]Rasmussen syndrome[/b] (with milia in addition).[/color][/color]

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[color="#000000"]Tumours develop from undifferentiated germinative cells of the pilosebaceous–apocrine unit and this explains why in some cases the same tumours have features of [/color][url="http://dermimages.med.jhmi.edu/images/cylindroma_2_060327.jpg"][color=windowtext]cylindroma[/color][/url][color="#000000"] and/or spiradenoma, particularly in the Brooke–Spiegler syndrome; there may be an admixture with trichoepitheliomas, either in separate tumours or sometimes in the same tumour. There is an apocrine line of differentiation at least in a percentage of spiradenomas (generally considered to be of eccrine sweat gland origin but Ackermann regarded it as apocrine in type and it is one of the painful skin tumours). Mutations in CLYD (cylindromatosis) gene, encoding for a tumour suppressor gene mapped to chromosome 16q12–13, may result in one of 3 syndromes: Brooke-Spiegler syndrome (epithelioma adenoides cysticum), familial cylindromatosis, and familial trichoepitheliomas.[/color][color=black][/color]

Cylindromas

Cylindroma

Cylindroma

Well done everyone. This is a cylindroma which as some of you have suggested might be associated with the Brooke-Spiegler syndrome. If my memory serves me correctly, this was the case in this specimen. Many thanks to Amr for such a comprehensive and valuable comment. I fully agree that there can be considerable overlap between spiradenoma and cylindroma.

Thank you Dr McKee.

Cylindroma

Thanks a lot for the teaching. I have the impression (from atlas) that the islands in cylindroma is more "jigsaw".

Thanks a lot again.

Thank you Dr Lee.