Desmoplastic Fibroma of Bone

  • Any bone can be involved.

  • Most cases occur in the jaw; metaphysis of long bones, especially the distal end of the radius; scapula; and pelvis.

Clinical Symptoms and Signs

  • Local pain and/or the presence of a mass is the usual initial symptom.

  • Pathological fracture occurs in between 12 and 20 % of the cases at presentation.

Image Diagnosis

Radiographic Features

  • Radiographs show a lytic, usually well-defined lobulated lesion, with no peripheral sclerosis or periosteal reaction. The bone can be slightly expanded, and with tumor growth, the cortex can be destroyed with soft tissue expansion of the lesion.

CT Features

  • CT confirms the absence of bone production by the tumor tissue.

MRI Features

  • MRI imaging usually presents iso- or hypointense signal relative to the adjacent muscle on T1- and a characteristic low signal on T2-weighted images.

  • CT and MRI help define the soft tissue extension of the lesion.

Image Differential Diagnosis

Chondromyxoid Fibroma

  • CMF has a better defined border inside the bone; expansion to soft parts shows a thin mineralized shell of reactive bone; MRI shows heterogeneous, predominantly high intensity signal on T2-weighted images.

Fibrous Dysplasia

  • It may be difficult to differentiate FD cases with scarce osteoid production.

Low-Grade Central Osteosarcoma

  • Usually presents blurred borders due to permeation into the host bone; variable bone production identified by radiographs, CT, or MRI

Fibrosarcoma of Bone

  • Has blurred borders due to permeative pattern; rarely expands the host bone


Gross Features

  • The cut surface is white and firm, with a whorled pattern and clear, scalloped margins.

Histological Features

  • More commonly, desmoplastic fibroma has a general low cellularity and abundant collagen matrix, with occasional permeation of neighboring tissues.

  • Cells are spindle and arranged in a whorled fashion or in long fascicles, similar to what happens in soft tissue desmoplastic fibroma. Nuclei look active and may show minimal atypia, and mitoses are absent or rare.

  • Long, dilated vascular channels are commonly seen.

  • Thick, undulating collagen fibers resembling keloid may be focally seen.

  • Reactive bone trabeculae may be focally present in a fractured or recurrent tumor, raising a differential diagnosis with fibrous dysplasia.

Pathology Differential Diagnosis

Fibrous Dysplasia

  • Presents a variable production of curvilinear immature bone trabeculae regularly disposed inside a slightly whorled and loose collagenic stroma; cellularity is low, it is regularly distributed, and cells are always typical; mitoses are absent.

Low-Grade Central Osteosarcoma

  • Has a permeative pattern; contains neoplastic bone trabeculae; cells are elongated, and although mitoses may be rare, nuclei have a slight atypical appearance. Positive for CDK4 and MDM2

Fibrosarcoma of Bone

  • Has a permeative pattern; more cellular and atypical than desmoplastic fibroma with a herringbone arrangement; occasional mitoses are seen, typical and atypical.

Ancillary Techniques

  • MDM2 and CDK4 are negative in desmoplastic fibroma and can differentiate it from low-grade osteosarcoma which will show positivity for these markers.

  • Beta-catenin is usually positive in the cytoplasm of neoplastic cells.


  • Rearrangement involving chromosomes 11 and 19 was reported.

Jan 2, 2017 | Posted by in ORTHOPEDIC | Comments Off on Desmoplastic Fibroma of Bone

Full access? Get Clinical Tree

Get Clinical Tree app for offline access