Desmoplastic fibroma

Dr Henry Knipe and Dr Yuranga Weerakkody et al.

Desmoplastic fibromas are extremely rare bone tumours that do not metastasise but may be locally aggressive. They are considered to be a bony counterpart of soft tissue desmoid tumours and are histologically identical. 

Incidence is ~0.3%. The most common areas of involvement include the mandible, pelvis and femur 2. Mean age at presentation is 21, and there is no sex predilection.

Desmoplastic fibromas histologically are identical to soft tissue desmoid tumours, with abundant collagenous stroma and little cellularity or pleomorphism. The main cell types that are seen include fibroblasts, myofibroblasts, and undifferentiated mesenchymal cells 9.

  • typically seen as a lytic bone lesion with a geographic pattern of bone destruction
  • often has a narrow zone of transition and non-sclerotic margins
  • internal pseudotrabeculation: >90% 3
  • no matrix mineralisation
  • widening of the host bone from gradual apposition of periosteal new bone formation: ~90% 3  

There is considerable overlap with other bony lesions on MR appearances. Signal characteristics include:

  • T1: typically low signal
  • T2: has background intermediate to high signal with intrinsic low to intermediate intensity foci within 7
  • T1 C+ (Gd): often shows heterogeneous enhancement 

Despite being benign it is a still locally aggressive tumour. En bloc resection is the current treatment of choice.  

Initially described by H. L Jaffe in 1958 4.

Bone tumours

The differential diagnosis for bone tumours is dependent on the age of the patient, with a very different set of differentials for the paediatric patient.

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Article information

rID: 10810
Tag: cases
Synonyms or Alternate Spellings:
  • Desmoid tumour of bone

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