Soft tissue
Fibroblastic / myofibroblastic
Sclerosing epithelioid fibrosarcoma

Topic Completed: 1 June 2013

Minor changes: 9 September 2020

Copyright: 2002-2020,, Inc.

PubMed Search: Sclerosing epithelioid fibrosarcoma [title]

H. Cliff Sullivan, M.D.
Jerad M. Gardner, M.D.
Page views in 2019: 4,014
Page views in 2020 to date: 3,231
Cite this page: Sullivan C. Sclerosing epithelioid fibrosarcoma. website. Accessed September 26th, 2020.
Definition / general
  • Rare, slow growing, sarcoma of deep soft tissue with epithelioid tumor cells in nests and cords set in hyalinized fibrous stroma
  • WHO Classification: Fibroblastic/Myofibroblastic Tumors
  • First described in 1995 (Am J Surg Pathol 1995;19:979)
Clinical features
Prognostic factors
  • Location appears to be linked to prognosis (Clin Orthop Relat Res 2008;466:1485):
    • Head and neck - 46% dead of disease
    • Upper extremity - 38% dead of disease
    • Trunk - 26 % dead of disease
  • No effect on prognosis:
    • Gender - no effect on local recurrence
    • Tumor size - metastatic disease independent of tumor size
Case reports
Gross description
  • Mean size: 9 cm (range 4 - 22 cm)
  • Grossly well circumscribed
  • Lobulated, bosselated, or multinodular
  • Firm, gray-white cut surface, occasionally cystic, calcified, myxoid
  • May invade bone
  • Usually no necrosis
Gross images

Contributed by Mark R. Wick, M.D.

Images hosted on other servers:

Lobulaulated, multinodular white mass

Microscopic (histologic) description
  • Low power: hypocellular tumor with large areas of hyalinized fibrous stroma
  • Mid power: nests and cords of small to medium sized, round to ovoid, relatively uniform epithelioid cells; some tumors have a more alveolar pattern; may have areas resembling fibroma, low grade fibromyxoid sarcoma, conventional fibrosarcoma; occasionally tumors have hemangiopericytoma-like vasculature, vascular invasion, necrosis (Histopathology 1998;33:354)
  • High power: often clear to scant eosinophilic cytoplasm and uniform bland nuclei with vesicular or finely stipple chromatin and small basophilic nucleoli; mitoses are inconspicuous, < 4 MF / 10 HPF
Microscopic (histologic) images

Contributed by Mark R. Wick, M.D.

Images hosted on other servers:

Alveolar pattern

Cytology description
  • Only one case report (Diagn Cytopathol 2010;38:748): clusters of moderately overcrowded epithelioid cells with background of histiocytes and lymphocytes and a few mesothelial cells
  • Nuclei eccentrically placed and pleomorphic with oval, spindled, or cleaved shape
  • Uniformly fine, granular chromatin and thin nuclear membrane
  • Numerous small nucleoli
  • Occasional multinucleated cells
Positive stains
Negative stains
Electron microscopy description
Molecular / cytogenetics description
Differential diagnosis
Back to top
Image 01 Image 02