Malignant Cutaneous Neoplasms with Peripheral Nerve Differentiation

  • Zsolt ArgenyiEmail author
  • Chris H. Jokinen
Part of the Current Clinical Pathology book series (CCPATH)


Malignant peripheral nerve sheath tumor (MPNST) of the skin is a rare superficial sarcoma, capable of locally destructive growth and systemic metastasis. By definition, MPNST arises from a preexisting peripheral nerve sheath tumor or a nerve de novo. In the former circumstance, nearly all arise from a neurofibroma with a high association with the plexiform variant. Rare deeper variants of MPNST with epithelioid cytology may arise from schwannoma; however, this has not been well established in cutaneous forms. MPNST shows evidence of schwannian differentiation by immunohistochemical or ultrastructural methods. Rare forms with perineurial differentiation (malignant perineurioma) are described in soft tissues but so far not in the skin.


Malignant peripheral nerve sheath tumors Malignant schwannoma Malignant granular cell tumor Classification Histogenesis Diagnostic features Differential diagnosis 

Additional Reading

Malignant Peripheral Nerve Sheath Tumor

  1. George E, Swanson PE, Wick MR. Malignant peripheral nerve sheath tumors of the skin. Am J Dermatopathol. 1989;11:213.CrossRefPubMedGoogle Scholar
  2. Thomas C, Somani N, Owen LG, Malone JC, Billings SD. Cutaneous malignant peripheral nerve sheath tumors. J Cutan Pathol. 2009;36:896.CrossRefPubMedGoogle Scholar
  3. Tomas D, Franjić DB, Mijić A, Kruslin B. Malignant peripheral nerve sheath tumor with numerous signet-ring and lipoblast-like cells. J Cutan Pathol. 2009; 36:77.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.SeattleUSA
  2. 2.Department of PathologyEssentia Health, Duluth ClinicDuluthUSA

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