Fibromatoses

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Micrograph showing a palmar fibromatosis. H&E stain.

Fibromatoses are a group of benign stromal lesions that may be seen in pediatric pathology and may or may not be associated with syndromes.

Syndromes associated with fibromatoses

Types

Superficial

Deep

Gross

  • Firm.
  • Infiltrative borders.

Image:

Microscopic

Features:

  • Bland spindle cells - typically in fascicles.
    • Pale eosinophilic cytoplasm.[2]
    • Nucleus may be ovoid.[3]
  • Small thin blood vessels that are parallel to one another.
    • Nuclei of blood vessels are typically darker staining than those of the lesion.

DDx:

Images:

IHC

  • Beta-catenin +ve -- nuclear stain.
  • CD117 -ve.

Image:

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SOFT TISSUE LESION, LEFT SHOULDER, CORE BIOPSY:
- FIBROMATOSIS, SEE COMMENT.

COMMENT:
The lesion was evaluated with immunostains:
Positive: vimentin, SMA (rare), beta-catenin.
Negative: pankeratin, S-100, desmin, CD34 (stains vessels).

Micro

The sections show a lesion with bland spindle cells. There is no apparent nuclear atypia. Mitotic figures are not identified. No myxoid areas are apparent. No necrosis is identified.

See also

References

  1. Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1092. ISBN 978-1416031215.
  2. URL: http://www.histopathology-india.net/Fibromatosis.htm. Accessed on: 18 September 2012.
  3. 3.0 3.1 3.2 Murphey, MD.; Ruble, CM.; Tyszko, SM.; Zbojniewicz, AM.; Potter, BK.; Miettinen, M. (Nov 2009). "From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation.". Radiographics 29 (7): 2143-73. doi:10.1148/rg.297095138. PMID 19926768.

External links