Virchows Archiv

, Volume 447, Issue 4, pp 677–682 | Cite as

Reticular and plexiform perineurioma: clinicopathological and immunohistochemical analysis of two cases and review of perineurial neoplasms of skin and soft tissues

  • Thomas Mentzel
  • Heinz Kutzner
Review Article


Perineurioma represents a recently described and relatively rare neoplasm in the spectrum of benign peripheral nerve sheath tumours composed of perineurial cells staining immunohistochemically positive for epithelial membrane antigen (EMA). In addition to intraneural, extraneural and sclerosing perineurioma, rare variants of perineurioma may occur, and their knowledge is important in the differential diagnosis of mesenchymal tumours of different lines of differentiation and clinically more aggressive neoplasms. We present a case of deep-seated reticular perineurioma arising on the upper arm of a 34-year-old female and a case of a dermal plexiform perineurioma arising on the lower lip of a 60-year-old female. The diagnosis was confirmed in both cases immunohistochemically; neoplastic cells stained positively for EMA and for the newly described perineurial markers, claudin-1 and glut-1. The morphological spectrum and the differential diagnosis of perineurial neoplasms of skin and soft tissues are discussed.


Nerve sheath neoplasms Perineurioma Epithelial membrane antigen Claudin-1 Human erythrocyte glucose transporter (glut-1) 



The authors are grateful to Dr. Outrata, Stuttgart, Germany, for kindly providing case material and clinical follow-up for case 1.


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Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  1. 1.Dermatopathologische GemeinschaftspraxisFriedrichshafenGermany

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