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cutaneous fibrous histiocytoma

Thursday 5 June 2003

Dermatofibroma, Sclerosing hemangioma, Histiocytoma cutis, Nodular subepidermal fibrosis, benign fibrous histiocytoma

Digital cases

- UI:923 - benign fibrous histiocytoma (dermatofibroma)
- PathConsult
- JRC:10461 : Dermatofibroma (fibrous histiocytoma) (Vs. gram cct)

Images

- pallisading dermatofibroma

- lipidized fibrous histiocytoma

- dermatofibroma with basaloid induction

Definition: Benign proliferation of dermal fibroblasts and histiocytes that generally presents as an asymptomatic, variably pigmented nodule, often on the extremities. Type of fibrohistiocytic tumors.

Clinical synopsis

- Firm
- Nodular
- Nonencapsulated
- Often pigmented
- Chiefly on extremities
- Single or multiple
- Flat, polypoid or depressed shape
- Most @<@1 cm diameter
- Some reach huge proportions

Pathogenesis

- Longstanding controversy as to whether:

  • neoplastic
    • favored by occurrence of aggressive and even metastasizing forms
    • evidence of clonality3–5
  • reactive

Gross Pathology

- Usually solid
- Rather well circumscribed
- Not encapsulated
- Colored white to yellow to dark brown depending on relative amounts of:

  • fibrous tissue
  • fat
  • hemosiderin

Histopathology

- Characteristically centered in upper dermis
- Can involve deep dermis
- Occasionally extends into subcutis
- Cellular fibroblastic proliferation
- Varying amounts of collagen deposition
- Variable number of macrophages

  • fat (foamy appearance)
  • hemosiderin (The tumor is mainly composed of hemosiderin-laden macrophages.)
  • some multinucleated cells
  • may acquire features of Touton’s giant cells
  • more rarely osteoclast-like features with or without bone formation.

- Fine vascular network:

  • can be prominent
  • responsible for: past diagnosis as sclerosing hemangioma
  • occasional misdiagnosis as Kaposi’s sarcoma, especially in HIV infection

- May be:

  • focal storiform features:
  • rarely as well developed as in dermatofibrosarcoma protuberans
  • smooth muscle proliferation within adjacent dermis

- Lesions blend imperceptibly into adjacent dermis

- Overlying epidermis:

  • normal, atrophic, or acanthotic
  • sometimes proliferation of hair germ-like structures in basal layer of epithelium
  • This lesion can be associated with basaloid proliferation of the overlying skin. (This change does not represent a basal cell carcinoma)
  • rarely basal cell carcinoma develops
  • exceptionally squamous cell carcinoma in situ

Morphologic variations (sometimes two or more coexist)

- prominent palisading similar to that in peripheral nerve tumors
- keloid-like changes
- myxoid changes
- granular cells
- markedly lipidized cells
- clear cells
- diffuse eosinophilic infiltrate
- lichenoid, erosive, and ulcerated features
- lipidized fibrous histiocytoma

Variants

- marked focal cellular atypia (manifested by ‘monster cells’)
- extreme cellularity (sometimes with necrosis)
- large cystic changes filled with blood: referred to as hemorrhagic aneurysmal or angiomatoid (distinguish from angiomatoid malignant fibrous histiocytoma seen in deeper sites in younger patients)
- epithelioid cell histiocytoma

  • mainly large ‘angulated’ epithelioid cells
  • notorious for simulating vascular and melanocytic neoplasms

- cellular fibrous histiocytoma
- aneurysmal fibrous histiocytoma
- atypical fibrous histiocytoma (pseudosarcomatous fibrous histiocytoma)
- epithelioid dermatofibroma (epithelioid fibrous histiocytoma)
- ossifying dermatofibroma with osteoclast-like giant cells
- metastasizing "benign" cutaneous fibrous histiocytoma (23426120)

Special Stains and Immunohistochemistry

- Proliferating spindle cells:

  • positive for: vimentin
  • usually negative for: lysozyme, other histiocytic markers (these results raise questions about alleged histiocytic nature)

- reactive for:

  • FXIIIa: a proenzyme in ‘dermal dendrocytes’

- negative for CD34 (in contrast with dermatofibrosarcoma protuberans)
- positive for tenascin

- Often markers associated with smooth muscle/myofibroblastic differentiation:

  • e.g. actin, desmin, myosin
  • not generally known; has led to misdiagnoses such as leiomyoma and leiomyosarcoma

Immunochemistry

- strong tenascin positivity at the dermal-epidermal junction overlying the lesion (100%)
- tenascin within the DF lesion (80%)
- CD34+ (25%) DF
- Factor XIIIa+ (95%) dermatofibrosarcoma

Differential Diagnosis

- melanocytic nevus
- Kaposi sarcoma
- malignant melanoma
- DFSP (11172295)

Marker dermatofibroma DFSP
CD34 25% 80%
FXIIIa 95% 15%
Tenascin in the tumor 80% 80%
Tenascin at the dermal-epidermal junction 100% 0%

- When heavily pigmented may be confused with:

  • melanocytic nevi
  • malignant melanoma
  • Kaposi sarcoma
  • other vascular tumors

Prognosis

- Generally indolent
- Local recurrence rare, even if margins inadequate
- Rarely:

  • locally aggressive

- Exceptionally rarely:

  • distant metastases
    • more common if: in face, deep extension into subcutaneous tissue or cellular fascicles of mitotically active spindle cells

See also

- fibrohistiocytic tumors

References

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