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Surgical Pathology Criteria
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Nuchal-type Fibroma

Definition

  • Hypocellular collagenous lesion with surrounded fat and nerves typically located in the subcutis of the posterior neck

Alternate / Historical Names

  • Collagenosis nuchae
  • Nuchal fibrocartilaginous pseudotumor
  • Nuchal fibroma

Diagnostic Criteria

  • Not encapsulated, radiates into fat
    • Frequent entrapped fat
  • Subcutaneous component in all cases
  • Paucicellular thick bundles of collagen
    • Few, small fibrocytes
  • Frequent entrapped nerves
    • May show traumatic neuroma-like fibrosis and proliferation
  • Infrequent infiltration of skeletal muscle
  • Typically located in or near posterior neck
  • Similar lesions occuring in children or extra-nuchal sites may be associated with Gardner syndrome

     

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

    Original posting: November 20, 2007
    Last Update: January 14, 2008

    Supplemental studies

    Immunohistology

    CD34 Positive
    CD99 Positive
    Smooth muscle actin Negative
    S100 Stains entrapped nerves

    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

     

    Nuchal-type Fibroma Fibrolipoma
    Radiates into fat Encapsulated
    Abundant collagen Focal collagen
    Frequent entrapped nerves Lacks entrapped nerves

     

    Nuchal-type Fibroma Elastofibroma
    Elastic fibers not prominent Prominent abnormal elastic fibers
    Subcutaneous component in all cases Deep, subscapsular

     

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

     

    Nuchal-type Fibroma Solitary Fibrous Tumor
    Paucicellular May have cellular areas
    Radiates into fat Usually circumscribed
    Inconspicuous vessels Frequent stag horn vessels

     

    Nuchal-type Fibroma Spindle Cell Lipoma
    Radiates into fat Encapsulated
    Abundant collagen Focal collagen
    Frequent entrapped nerves Lacks entrapped nerves

     

    Nuchal-type Fibroma Scar
    No history of trauma History of trauma
    These two may be histologically indistinguishable

     

    Nuchal-type 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
    Most age 20 to 40, unless associated with Gardner syndrome Age range congenital to 4 years
    Typically located in or near posterior neck Usually axillary or inguinal

    Clinical

    • Age range 3-74 years
    • Predominantly male
    • Predominantly posterior neck but also in other sites
    • Similar lesions occuring in children or extra-nuchal sites may be associated with Gardner syndrome
      • This may be the presenting sign of Gardner syndrome
    • Lesions not associated with Gardner syndrome may recur but not aggressively
    • Lesions associated with Gardner syndrome may recur as desmoid fibromatosis
    • 44% of cases in series of Michal had diabetes

    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
    • 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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