Bone & joints

Fibrogenic / fibrohistiocytic tumors

Desmoplastic fibroma of bone



Last author update: 1 June 2005
Last staff update: 12 October 2023

Copyright: 2003-2024, PathologyOutlines.com, Inc.

PubMed Search: Desmoplastic fibroma bone

Nat Pernick, M.D.
Page views in 2023: 5,078
Page views in 2024 to date: 2,733
Cite this page: Pernick N. Desmoplastic fibroma of bone. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/bonedesmoplasticfibroma.html. Accessed May 19th, 2024.
Definition / general
  • Rare, benign / borderline behavior; bony counterpart of soft tissue fibromatosis
  • Intraosseous component of soft tissue fibromatosis
  • May be due to local trauma; may be part of Gardner syndrome
  • Benign but up to 35% recur, does not metastasize
Epidemiology
  • Mean 23 years, range 1 - 75 years
  • 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, nonencapsulated, fibrous rubbery mass with variable bony spicules and cysts
Microscopic (histologic) description
  • Poorly demarcated lesion with interlacing or fascicular pattern of mature fibrous tissue composed of small fibroblasts with no / minimal mitotic activity and abundant collagenous stroma
  • Mature, bland fibroblasts separated by abundant collagen with thin walled, dilated vascular channels
  • May infiltrate into soft tissue
  • No necrosis, no pleomorphism or atypia
Microscopic (histologic) images

Contributed by Kelly Magliocca, D.D.S., M.P.H.
Desmoplastic fibroma

Desmoplastic fibroma

Molecular / cytogenetics description
  • Trisomy 8, trisomy 20
Positive stains
Negative stains
Electron microscopy description
  • Predominantly myofibroblasts, also fibroblasts and primitive mesenchymal cells
Additional references
Back to top
Image 01 Image 02