Stanford School of Medicine
Surgical Pathology Criteria
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Proliferative Fasciitis

Definition

  • Subcutaneous or fascial reactive process composed of large ganglion-like cells, fibroblasts and myofibroblasts set in a myxoid stroma

Diagnostic Criteria

  • Predominantly involves subcutaneous tissue
    • May appear grossly infiltrative
    • May extend along fascia
  • Generally loose myxoid stroma
    • May contain collagen
      • May hyalinize
    • May have central arborizing vascular pattern
    • Small vessels often arranged in clusters or in a linear fashion
  • Spindled and stellate fibroblasts and myofibroblasts
    • Vesicular nuclei
    • No cytologic atypia
  • Ganglion-like cells
    • Characteristic feature of this process
    • Large round regular nuclei
    • Occasional binucleate and trinucleate cells may be seen
    • Prominent nucleoli
    • Dispersed chromatin
    • Basophilic to amphophilic cytoplasm
    • May be clustered and may mold each other
  • Mitotic figures frequent
    • No atypical mitotic figures
  • Rare lesions in children have some differences
    • Reported age range 2 months to 13 years
    • Better circumscription
    • Lobulated
    • More cellular
    • More ganglion-like cells, fewer fibroblasts
    • May show focal necrosis and acute inflammation
  • Richard L Kempson MD
    Robert V Rouse MD
    Department of Pathology
    Stanford University School of Medicine
    Stanford CA 94305-5342

    Original posting: March 6, 2008
    Last update: June 15, 2008

 

Supplemental studies

Immunohistology

  • Ganglion-like cells
    • Factor XIIIa positive
    • Actin negative to focal/weak
    • Desmin, S100, neuron specific enolase negative
  • Spindled/stellate cells
    • Actin positive
    • CD68 variable
    • S100 negative

Differential diagnosis

Proliferative Myositis and Ischemic Fasciitis differ from Proliferative Fasciitis only by location and clinical presentation

 

Proliferative Fasciitis Nodular Fasciitis
Ganglion-like cells present No ganglion-like cells
Clusters or linear arrangement of small vessels Vessels usually not clustered

 

Fibromatosis, Abdominal Desmoid and Extra-abdominal Desmoid Proliferative Fasciitis
No ganglion-like cells Ganglion-like cells present
Usually >3 cm Usually <4 cm
Predominantly collagenous Predominantly myxoid
Almost always involves muscle with degenerating muscle cells at tumor border Usually does not involve muscle

 

Ganglioneuroma Proliferative Fasciitis
Fibrillar background Myxoid background with reactive myofibroblasts
S100 may stain satellite cells S100 negative

 

Malignant Neoplasm, NOS Proliferative Fasciitis
Cytologically malignant Lacks malignant cytologic features
Atypical mitotic figures may be present No atypical mitotic figures
Usually >4 cm Usually <4 cm

 

Rhabdomyosarcoma Proliferative Fasciitis
May have cross-striations No cross-striations
Cytooplasm typically eosinophilic Cytoplasm typically basophilic
Desmin, myogenin positive Desmin, myogenin negative
Cytologically malignant Lacks malignant cytologic features
Atypical mitotic figures may be present No atypical mitotic figures
Usually >4 cm Usually <4 cm

Clinical

  • Usually middle aged or older
    • Rare cases reported in children, ages 2 months to 13 years
  • Usually short duration
  • Usually forearm or thigh
  • Recurrence following excision is quite rare

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
  • Lundgren L, Kindblom LG, Willems J, Falkmer U, Angervall L.  Proliferative myositis and fasciitis. A light and electron microscopic, cytologic, DNA-cytometric and immunohistochemical study.  APMIS 1992 May;100(5):437-48
  • Ushigome S, Takakuwa T, Takagi M, Koizumi H, Morikubo M.  Proliferative myositis and fasciitis. Report of five cases with an ultrastructural and immunohistochemical study.  Acta Pathol Jpn 1986 Jul;36(7):963-71
  • Meis JM, Enzinger FM.  Proliferative fasciitis and myositis of childhood.  Am J Surg Pathol 1992 Apr;16(4):364-72.
  • Chung EB, Enzinger FM.  Proliferative fasciitis.  Cancer 1975 Oct;36(4):1450-8.
 
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