Stanford School of Medicine
Surgical Pathology Criteria
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Giant Cell Fibroblastoma

Definition

  • Superficial infiltrative soft tissue neoplasm characterized by irregular pseudovascular spaces lined by pleomorphic giant cells

Diagnostic Criteria

  • Poorly circumscribed superficial soft tissue mass
    • Most cases localized to subcutaneous tissue
      • Occasional involvement of dermis
      • Rare involvement of superficial underlying skeletal muscle
      • No deep visceral involvement reported
    • Infiltrates between and around fat cells and adnexa
      • Produces “string of pearls” or “honeycomb” or “lace-like” patterns
  • Myxoid to hyalinized stroma with variable cellularity
  • Two cell populations
    • Small bland spindled to stellate cells
      • Mild to moderate pleomorphism at most
    • Scattered multinucleated giant cells
      • (Ultrastructure shows that they are actually just multilobated)
      • Vesicular nuclei with prominent nucleoli
    • Infrequent mitotic figures
  • Irregular pseudovascular angiectoid spaces
    • Partially lined by small stromal cells and giant cells
    • No endothelial lining
  • Dermatofibrosarcoma protuberans is a closely related lesion
    • More common in adult age group
    • Mixtures may be seen
    • Recurrences may be of the other neoplasm
    • Same (17;22) translocation
  • Fibrosarcoma has not been reported in pure giant cell fibroblastoma
    • May be seen in cases with a component of dermatofibrosarcoma protuberans
  • Occasional findings:
    • Perivascular onion skin-like lymphocytic infiltrate
    • Intralesional hemorrhage
    • Myoid whorls
      • Small collections of actin positive cells with abundant cytoplasm

    • 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

CD34 Nearly 100%, may be weak
CD31 Negative
Actin Negative except for myoid whorls
Keratin Negative
S100 Negative
Desmin Negative

Genetic studies

t(17;22) with fusion of COL1A1 and PDGFB

Differential diagnosis

Fibrous Hamartoma of Infancy Giant Cell Fibroblastoma
Triphasic with mature spindled fibroblasts, nodules of immature cells and fat Lacks triphasic pattern
Lacks giant cells Prominent giant cells
Lacks pseudovascular spaces Prominent pseudovascular spaces

 

Angiosarcoma Giant Cell Fibroblastoma
Cytologically atypical Giant cells appear multinucleated rather than cytologically atypical
Anastomosing vascular channels Irregular pseudovascular spaces
Tufted endothelial growth is frequent Lacks tufted pattern
May be mitotically active Mitotic figures rare
Spaces lined by CD31 positive endothelium CD31 negative
Usually occurs in sun exposed skin of adults, rare in children Mean age 3-6 years

 

Myxoid Liposarcoma Giant Cell Fibroblastoma
Always involves deep soft tissue Usually involves superficial subcutaneous tissue
Frequently contains lipoblasts No lipoblasts
CD34 may stain scattered dendritic cells but not lesional cells CD34 stains lesional cells
Arborizing vascular pattern Pseudovascular spaces
Lacks giant cells Frequent giant cells

 

Plexiform Fibrohistiocytic Tumor Giant Cell Fibroblastoma
Ray-like extension of fibrous tissue into surrounding fat Infiltrates between and around individual fat cells
Lacks pseudovascular spaces Pseudovascular spaces present
Nodules of histiocytes and giant cells in most cases Scattered individual giant cells
CD34 negative CD34 stains lesional cells

 

Myxofibrosarcoma Giant Cell Fibroblastoma
Usually seen in adults, rare under 20 Mean age 3-6 years
Cytologically atypical but no multinucleated giant cells Giant cells appear multinucleated rather than cytologically atypical
May be mitotically active Mitotic figures rare
Lacks pseudovascular spaces Pseudovascular spaces present

 

Atypical Deep Fibrous Histiocytoma Giant Cell Fibroblastoma
Lacks pseudovascular spaces Pseudovascular spaces present
CD34 negative CD34 stains lesional cells

Clinical

  • Most cases localized to subcutaneous tissue
    • Occasional involvement of dermis
    • Rare involvement of superficial underlying skeletal muscle
    • No deep visceral involvement reported
  • May be seen at any site except hands and feet
    • Most common on trunk and thigh
  • Up to 50% recur
  • No metastases reported for pure lesions
  • Median age 3 to 6 years
    • Age 6 months to 62 years

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
  • Weiss SW, Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors, 5th edition, 2008
  • Shmookler BM, Enzinger FM, Weiss SW. Giant cell fibroblastoma. A juvenile form of dermatofibrosarcoma protuberans. Cancer. 1989 Nov 15;64(10):2154-61.
  • Dymock RB, Allen PW, Stirling JW, Gilbert EF, Thornbery JM. Giant cell fibroblastoma. A distinctive, recurrent tumor of childhood. Am J Surg Pathol. 1987 Apr;11(4):263-71.
  • Jha P, Moosavi C, Fanburg-Smith JC. Giant cell fibroblastoma: an update and addition of 86 new cases from the Armed Forces Institute of Pathology, in honor of Dr. Franz M. Enzinger. Ann Diagn Pathol. 2007 Apr;11(2):81-8.
  • Billings SD, Folpe AL. Cutaneous and subcutaneous fibrohistiocytic tumors of intermediate malignancy: an update. Am J Dermatopathol. 2004 Apr;26(2):141-55.
  • Terrier-Lacombe MJ, Guillou L, Maire G, Terrier P, Vince DR, de Saint Aubain Somerhausen N, Collin F, Pedeutour F, Coindre JM. Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children: clinicopathologic comparative analysis of 28 cases with molecular data--a study from the French Federation of Cancer Centers Sarcoma Group. Am J Surg Pathol. 2003 Jan;27(1):27-39.
 
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