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Skin - Nonmelanocytic tumors

Other tumors of skin

Neurothekeoma

Reviewer: Christopher Hale, M.D. (see Reviewers page)
Revised: 18 September 2012, last major update September 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● Also called nerve sheath myxoma
● Benign, despite atypia and mitotic figures
● Rarely recurs
● Children or teenagers with tumors of central face, arms and shoulders
● 80% female

Myxoid variant of neurothekeoma

Micro description
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● Encapsulated or sharply circumscribed, nests or cords of large epithelioid cells with mild/moderate atypia in myxoid or sclerotic background
● Tumor cells may be close to small nerves
● Variable mitotic figures

Positive stains
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● Usually NKI/C3 and microphthalmia transcription factor, type 4 collagen; variable S100

Negative stains
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● Usually EMA, keratin

Cellular variant of neurothekeoma

General
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● Head and neck of young women

Micro description
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● Dermal or subcutaneous proliferation of nests and fascicles of tumor cells filling and expanding superficial and deep dermis
● Cells are epithelioid with abundant eosinophilic or amphophilic cytoplasm and overlapping cell membranes
● Nuclear are vesicular with dispersed chromatin
● Variable hyperchromatism
● Also spindled cells
● No involvement of epidermis or junctional melanocytic proliferation
● No mucinous stroma, no maturation, no circumscription

Micro images
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Various images

Positive stains
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● NKI/C3, smooth muscle actin, microphthalmia transcription factor, PGP 9.5, NSE, Leu7 (Mod Pathol 2004;17:230)

Negative stains
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● S100, HMB45

Differential diagnosis
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● Melanoma

End of Skin - Nonmelanocytic tumors > Other tumors of skin > Neurothekeoma

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