Cutaneous Proliferations with Putative Neural Differentiation

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


Neurothekeoma (NTK) is a neoplasm of uncertain lineage. Previous descriptions classified NTK as cellular (immature) and myxoid (mature; classical) types based on the degree of cellularity. Currently, however, it is suggested that some highly myxoid and the previously described transitional or mixed variants with evidence of schwannian differentiation are better classified as dermal nerve sheath myxoma (see Chap.  4). NTK as currently classified on the other hand shows no convincing evidence of peripheral nerve lineage, and comprises a spectrum of tumors with variable degrees of cellularity and myxoid change. While the precise histogenesis is uncertain, some authors suggest this tumor may be of smooth muscle or fibrohistiocytic lineage. In general NTK is a benign neoplasm. While atypical forms exist, a true malignant variant has not yet been established.


Putative neural tumors Definitions Classifications Histogenesis Diagnostic features Differential diagnosis Neurothekeoma Merkel cell carcinoma Cutaneous neuroendocrine carcinoma Neurofollicular hamartoma 

Additional Reading


  1. Fetsch JF, Laskin WB, Hallman JR, Lupton GP, Miettinen M. Neurothekeoma: an analysis of 178 tumors with detailed immunohistochemical data and long-term patient follow-up information. Am J Surg Pathol. 2007;31:1103–14.CrossRefPubMedGoogle Scholar
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Primary Cutaneous Neuroendocrine Carcinoma

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Neurofollicular Hamartoma

  1. Barr RJ, Goodman MM. Neurofollicular hamartoma: a light microscopy and immunohistochemical study. J Cutan Pathol. 1989;16:336–41.CrossRefPubMedGoogle Scholar
  2. Sangueza OP, Requena L. Neurofollicular hamartoma: a new histogenetic interpretation. Am J Dermatopathol. 1994;16:150–4.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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