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Skin - Nonmelanocytic tumors

Other tumors of skin

Malignant peripheral nerve sheath tumor (MPNST)


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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● More common in deep soft tissue (see Soft Tissue chapter) but also present in skin
● May be associated with neurofibromatosis type I
● p53 expression may correlate with worse prognosis (Neuro Oncol 2009;11:514)

Case reports
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● Two NF-1 patients with MPNST (not necessarily cutaneous, Cases J 2009 Jun 9;2:7612)
● 49 year old man with cutaneous MPNST arising in neurofibroma, with lymph node metastasis (Int J Clin Exp Pathol 2010;3:812)

Micro description
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● Hypercellular, plexiform fascicles of neural cells, high mitotic activity, significant pleomorphism

Micro images
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Various images

Positive stains
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● S100, vimentin, CD56, CD117 (weak)

Molecular description
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● Numerous cytogenetic abnormalities
● Triploidy common
● Most frequent translocations are 7p22 and 1p21, 7p11 and 14q11 (not necessarily cutaneous tumors, Int J Cancer 1995;61:793)

End of Skin - Nonmelanocytic tumors > Other tumors of skin > Malignant peripheral nerve sheath tumor (MPNST)


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