Angiofibroma of soft tissue

Dr Joachim Feger et al.

Angiofibromas of soft tissue are benign fibroblastic soft tissue neoplasms permeated by a vascular network that might be found in the periarticular and articular areas of the lower extremities that have been included as a separate entity into the WHO classification of soft tissue tumors in 2020.

Angiofibromas of soft tissue tumors are mostly found in the middle-aged population with a peak incidence in the sixth decade and a slight female predominance. They can generally affect patients of all ages 1.

The typical clinical presentation is a slow-growing soft tissue mass 1.

Angiofibromas of soft tissue are characterized by uniform and spindle cells in a fibromyxoid matrix permeated innumerable small thin-walled blood vessels 1.

The etiology of angiofibroma of soft tissue is unknown 1.

Soft tissue angiofibromas are usually found in the extremities in particular the lower extremity with a periarticular articular involvement, a subcutaneous or less often intramuscular or deep location. Unusual sites of origin are the back, abdominal wall the pelvis and breast 1-4.

Macroscopically angiofibromas of soft tissue are usually solid nodular or multinodular well-delineated tumors with a shimmering cut surface and a white to yellowish color 1,4.

Histologically angiofibromas of soft tissue include the following microscopic features 1-4:

  • uniform bland spindle cells with regional variations in cellularity
  • variable myxoid to collagenous extracellular matrix
  • prominent vascular network
  • variable inflammatory infiltrates
  • no cytological atypia or nuclear hyperchromasia

Immunochemistry stains are variably positive for CD34, epithelial membrane antigen (EMA) and desmin 1,3,4.

Angiofibromas of soft tissue are associated with NCOA2 gene rearrangements 1-4.

Dedicated descriptions of imaging appearances of angiofibroma of soft tissue are still scarce in the literature. A few reports have described it as a well-circumscribed mass. Signal characteristics and enhancement are presumed to be variable subject to the cellular, myxoid, collageneous and vascular content of the lesion 3,4.

  • T1: roughly isointense to skeletal muscle
  • T2: heterogeneous high signal intensity
  • T1C+(Gd): variable from homogeneous to a peripheral enhancement

The radiological report should include a description of the following:

  • form, location and size
  • tumor margins and transition zone
  • relation to adjacent joints
  • relation to adjacent neurovascular structures

Angiofibromas of soft tissue are benign lesions with a benign clinical course. They have rare local recurrences after excision and there is no known risk for metastases 1,3.

Angiofibroma of soft tissue is a fairly newly described tumor entity by Adrián Mariño-Enríquez and Christopher DM  Fletcher in 2012 3-5.

Tumors or conditions which can mimic the presentation and/or appearance of angiofibroma of soft tissue include 3,4:

Article information

rID: 87289
Tag: cases3
Synonyms or Alternate Spellings:
  • Soft tissue angiofibroma
  • Angiofibromas of soft tissue

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