Soft tissue
Peripheral nerve

Topic Completed: 1 November 2012

Revised: 26 March 2019

Copyright: 2002-2018,, Inc.

PubMed Search: Neurothekeoma [title] soft tissue

Vijay Shankar, M.D.
Page views in 2018: 7,588
Page views in 2019 to date: 6,819
Cite this page: Shankar V. Neurothekeoma. website. Accessed September 18th, 2019.
Definition / general
  • Superficial tumor, first described in 1980
  • Originally, derivation thought to be from nerve sheath; now thought to derive from fibroblasts with ability to differentiate into myofibroblasts and to recruit histiocytes (Am J Surg Pathol 2007;31:1103)
  • Usually head, upper extremities or shoulder girdle
Clinical features
  • Solitary, superficial, slow growing mass up to 2 cm
  • 60% women, mean age 17 years (range 2 - 85 years), 80% are < age 30 at initial diagnosis
Case reports
  • Excision, may recur
Clinical images

Images hosted on other servers:

Thumb nodule

Lower eyelid

Lower back

Microscopic (histologic) description
  • Cellular, myxoid or mixed subtypes
  • Involves dermis or subcutis
  • Multinodular mass with myxoid matrix and peripheral fibrosis
  • Whorled or focally fascicular patterns of spindled and epithelioid mononuclear cells with abundant cytoplasm, indistinct cell borders
  • Margins usually positive; usually occasional multinucleated giant cells
  • Variable nuclear atypia
  • Median 4 MF / 25 HPF, may have 10+ MF / 25 HPF, may be atypical
Microscopic (histologic) images

Images hosted on PathOut servers:
Courtesy of Mark R. Wick, M.D.:


Cellular type

Cellular type, skin

Images hosted on other servers:

Left thumb nodule of 5 month old boy

Lower eyelid of elderly man

Lobular tumor proliferations with an abundant myxoid stroma

Mucin deposits, alcian blue


Positive stains
Negative stains
Differential diagnosis
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