Bone & joints

Fibrogenic / fibrohistiocytic tumors

Desmoplastic fibroma of bone



Topic Completed: 1 June 2005

Minor changes: 30 December 2020

Copyright: 2003-2021, PathologyOutlines.com, Inc.

PubMed search: desmoplastic fibroma [title] bone

Nat Pernick, M.D.
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Cite this page: Pernick N. Desmoplastic fibroma of bone. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/bonedesmoplasticfibroma.html. Accessed October 25th, 2021.
Definition / general
  • Rare, benign / borderline behavior; bony counterpart of soft tissue fibromatosis
  • 75% younger than age 30 years, may be more common in males
  • Sites: metaphysis of long bones (56%), mandible (26%), pelvis (14%)
Radiology description
  • Lytic and honeycombed (“soap bubble” appearance) metaphyseal lesions, cortical thinning with soft tissue extension
Case reports
Treatment
  • Wide local excision to prevent otherwise frequent recurrences
  • Causes local destruction, no metastases
Gross description
  • White gray, fibrous rubbery mass with variable bony spicules and cysts
Microscopic (histologic) description
  • Mature, bland fibroblasts separated by abundant collagen with thin walled, dilated vascular channels
  • May infiltrate into soft tissue
  • No necrosis, no pleomorphism or atypia, no mitotic activity
Molecular / cytogenetics description
  • Trisomy 8, trisomy 20
Electron microscopy description
  • Predominantly myofibroblasts, also fibroblasts and primitive mesenchymal cells
Additional references
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