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Surgical Pathology Criteria
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Gardner-associated Fibroma

Definition

  • Hypocellular collagenous lesion with surrounded fat similar to nuchal-type fibroma but occurring in children or in extra-nuchal sites

Diagnostic Criteria

  • Histologic features similar to nuchal-type fibroma
    • Not encapsulated, radiates into fat
      • Frequent entrapped fat
    • Subcutaneous component in all cases
    • Paucicellular formless sheets of collagen
      • Few, small fibrocytes
    • Rare entrapped nerves
      • Lacks traumatic neuroma like pattern
    • Infrequent infiltration of skeletal muscle
  • Most located on trunk
    • Also head and neck, extremites
  • May be associated with Gardner syndrome
    • Such lesions in extra-nuchal sites should prompt clinical investigation for Gardner syndrome
      • Found in 70%
      • This may be the presenting finding
  • Beta-catenin positive in 64%
  • Recurrence appears to be more common than with nuchal-type fibroma
  • Gardner syndrome
    • Familial adenomatous polyposis
    • Osteomas, especially of mandible
    • Mesenteric, and rarely abdominal, fibromatosis
    • Cutaneous epidermoid cysts

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

Original posting : November 20, 2007
Last Update: December 22, 2008

Differential Diagnosis

Gardner Associated Fibroma Nuchal-type Fibroma
Age range 2 mo to 36 years Age range 3 to 74 years
Most, but not all cases, extranuchal 70% nuchal
Formless sheets of collagen Thick bundles of collagen
Rare entrapped nerves Frequent entrapped traumatic neuroma areas
The two may be very difficult to distinguish histologically

Gardner Associated Fibroma Scar
No history of trauma History of trauma
These two may be histologically indistinguishable

Gardner Associated Fibroma Fibrolipoma
Radiates into fat Encapsulated
Abundant collagen Focal collagen

Gardner Associated Fibroma Fibrous Hamartoma of Infancy
Lacks triphasic pattern, no immature component Triphasic lesion with spindle cell fibrous areas, adipose tissue and clusters of immature mesenchymal cells
Age range 2 mo to 36 years All but rare cases congenital to 4 years

Gardner Associated Fibroma Extra-abdominal Desmoid Fibromatosis
Involves superficial tissues Involves deep tissues
Infrequently involves skeletal muscle Involves skeletal muscle
Paucicellular May have cellular areas

Gardner Associated Fibroma Solitary Fibrous Tumor
Paucicellular May have cellular areas
Radiates into fat Usually circumscribed
Inconspicuous vessels Frequent stag horn vessels

Lists

Soft tissue "fibromas"

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, 4th edition, 2001
  • Zamecnik M, Michal M.  Nuchal-type fibroma is positive for CD34 and CD99.  Am J Surg Pathol 2001 Jul;25(7):970 
  • Wehrli BM, Weiss SW, Yandow S, Coffin CM.  Gardner-associated fibromas (GAF) in young patients: a distinct fibrous lesion that identifies unsuspected Gardner syndrome and risk for fibromatosis.  Am J Surg Pathol 2001 May;25(5):645-51
  • Michal M, Fetsch JF, Hes O, Miettinen M.  Nuchal-type fibroma: a clinicopathologic study of 52 cases.  Cancer 1999 Jan 1;85(1):156-63
  • Balachandran K, Allen PW, MacCormac LB.  Nuchal fibroma. A clinicopathological study of nine cases.  Am J Surg Pathol 1995 Mar;19(3):313-7
  • Gardner EJ, Richards RC. Multiple cutaneous and subcutaneous lesions occurring simultaneously with hereditary polyposis and osteomatosis. Am J Hum Genet. 1953 Jun;5(2):139-47.
  • Coffin CM, Hornick JL, Zhou H, Fletcher CD. Gardner fibroma: a clinicopathologic and immunohistochemical analysis of 45 patients with 57 fibromas. Am J Surg Pathol. 2007 Mar;31(3):410-6.

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