Stanford School of Medicine
Surgical Pathology Criteria
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Desmoplastic Fibroblastoma

Definition

  • Densely collagenous lesion with low cellularity composed of spindled to stellate fibroblasts

Alternate / Historical Names

  • Collagenous fibroma

Diagnostic Criteria

  • May be subcutaneous or intramuscular
  • Grossly well circumscribed
    • Microscopically frequently infiltrates fat and muscle focally
    • May be lobulated
  • Size range 1-20 cm, median 3-4 cm
  • Sparse spindled to stellate large fibroblasts
    • Cytologically bland
  • Fibromyxoid to densely fibrous background
    • Hypovascular stroma
  • Mitotic figures rare to absent
  • No calcification or necrosis

Richard L Kempson MD
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting: August 10, 2008

Supplemental studies

Immunohistology

Vimentin positive
Smooth muscle actin focal positive in some cases
Keratin rare positive in few cases
S100 diffuse faint in few cases
CD34, Desmin, EMA negative
 

Genetic Study

Clonal rearrangement of 11q22 reported in two cases studied

Differential diagnosis

Fibromatosis, Abdominal Desmoid and Extra-abdominal Desmoid Desmoplastic Fibroblastoma
Mild to moderate cellularity Paucicellular
Moderate vascularity Hypovascular
Extensive infiltration of muscle Only focal muscle infiltration
Spindle cells usually in fascicular pattern Spindle and stellate cells randomly aranged

 

Desmoplastic Fibroblastoma Fibroma of Tendon Sheath
Wide distribution Most on hands or wrist
Single nodule Multilobular
Hypovascular Moderately vascular

 

Nuchal-type Fibroma Desmoplastic Fibroblastoma
Small cells with small nuclei Large cells with large nuclei
Poorly circumscribed Grossly well circumscribed

 

Desmoplastic Fibroblastoma Calcifying Fibrous Pseudotumor
No calcification Calcification present
Large stellate cells Small spindled cells
Inflammation uncommon Chronic inflammation present

 

Desmoplastic Fibroblastoma Nodular Fasciitis, Hyalinized
Paucicellular Moderately cellular areas
No mucin Cystic mucin pools, tissue tears
Lacks S and C shaped bundles of cells S and C shaped bundles of cells
Mitotic figures absent or rare Mitotic figures may be numerous

 

Low Grade Fibromyxoid Sarcoma Desmoplastic Fibroblastoma
Moderately cellular Paucicellular
Small nuclei, dense chromatin Large vesicular nuclei
Myxoid areas No myxoid areas
Whorled pattern No pattern

Clinical

  • Age range 16-81
    • Most 40-70
  • Painless, slow growing
  • Wide variety of sites
  • No recurrences

Bibliography

  • Kempson RL, Fletcher CDM, Evans HL, Henrickson MR, Sibley RS. Tumors of the Soft Tissues, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 30, 2001
  • Fletcher CDM, Unni KK, Mertens F. Pathology and Genetics of Tumours of Soft Tissue and Bone, World Health Organization Classification of Tumours 2002
  • Weiss SW, Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors, 5th edition, 2008
  • Evans HL.  Desmoplastic fibroblastoma. A report of seven cases.  Am J Surg Pathol 1995 Sep;19(9):1077-81.
  • Nielsen GP, O'Connell JX, Dickersin GR, Rosenberg AE.  Collagenous fibroma (desmoplastic fibroblastoma): a report of seven cases.  Mod Pathol 1996 Jul;9(7):781-5.
  • Miettinen M, Fetsch JF.  Collagenous fibroma (desmoplastic fibroblastoma): a clinicopathologic analysis of 63 cases of a distinctive soft tissue lesion with stellate-shaped fibroblasts.  Hum Pathol 1998 Jul;29(7):676-82.
  • Sciot R, Samson I, van den Berghe H, Van Damme B, Dal Cin P.  Collagenous fibroma (desmoplastic fibroblastoma): genetic link with fibroma of tendon sheath?  Mod Pathol 1999 Jun;12(6):565-8.
 
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