Skin tumor - Nonmelanocytic - Acrosyringeal adenomatosis

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Skin - Nonmelanocytic tumors

Adnexal tumors - eccrine sweat glands

Acrosyringeal adenomatosis

Reviewer: Christopher Hale, M.D. (see Reviewers page)
Revised: 23 June 2012, last major update June 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● Also called eccrine syringofibroadenoma (of Mascaro)
● Mostly older adults, acral
● Gradual and symmetric spread of papular lesions over large areas of body; occasional association with neoplastic or inflammatory conditions
● Shopfe-Schultz-Passarge syndrome: autosomal dominant with eccrine syringofibroadenoma, palmoplantar keratoderma, hypodontia, hidrocystomas (Acta Derm Venereol 2008;88:607)

Case reports
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● 63 year old man with diffuse asymptomatic papules (Am J Dermatopathol 1992;14:328)

Gross description
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● Verrucous papules, nodules

Micro description
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● Thin anastomosing cords/strands of monomorphous cuboidal cells extending from epidermis into dermis
● Loose fibrovascular stroma

Micro images
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Numerous thin, anastamosing strands of epithelium coming off multiple points of epidermis; they are 2-3 cells in thickness, with background of fibromucinous stroma

There are scattered ductules within the epithelium, containing bland cells with no peritumoral clefting; stroma is fibromucinous and mildly hypercellular

Electron macroscopy description
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● Tumor cells with tonofilaments/desmosomes, numerous glycogen granules
● Basal lamina
● Globular keratohyaline granules around ducts
● No lamellar granules
● Poorly developed cornified cell envelopes (Am J Dermatopathol 1996;18:207)

Positive stains
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● EMA, CEA, CK6, CK19, filaggrin

Differential diagnosis
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● Fibroepithelioma of Pinkus

End of Skin - Nonmelanocytic tumors > Adnexal tumors - eccrine sweat glands > Acrosyringeal adenomatosis

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