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Dermatofibrosarcoma Protuberans (DFSP)

DEFINITION AND PATHOGENESIS

  • A nodular cutaneous low-grade infiltrative malignant tumor with a high propensity for local recurrences

IMPORTANCE

  • Should be recognized for its aggressiveness to avoid recurrences and potential metastases
  • 6% of all soft tissue sarcomas

CLINICAL FEATURES

  • Nodular cutaneous mass
  • Early or mid-adult life
  • M>F
  • Trunk and proximal extremities
  • History of trauma in 10-20%
  • Reported to occur within previous scar or burn
  • Firm plaque-like skin lesion initially, progressing to a protuberant multinodular mass
  • ? surrounding red to blue discoloration
  • ? satellite nodules, esp in large lesions

RADIOLOGIC FEATURES

  • CT: unmineralized nodular mass involving skin and subcutaneous fat
    • Well-defined lesion with distinct lobular or nodular architecture and tissue attenuation equal or greater than muscle
    • Moderate enhancement
  • MRI best at delineation of size and depth of involvement
    • Nonspecific appearance
    • T1-weighted images with low signal intensity, isointense to muscle
    • T2-weighted images with high signal intensity

GROSS PATHOLOGY

  • Solitary protuberant, grayish, infiltrative mass involving skin and/or subcutaneous tissue

HISTOLOGIC/MOLECULAR FEATURES

  • Diffuse infiltration of dermis and subcutaneous tissue
  • Bland border of advancing spindled, fibroblast-like cells interdigitating with fat and adnexal tissues
  • Uniform plump fibroblasts in monotonous storiform pattern, in a pinwheel configuration around vessels
  • Low to moderate nuclear mitotic rate
  • Little collagen
  • ± multinucleated giant cells
  • Has a t(17;22) translocation resulting in deregulated expression of platelet-derived growth factor B
    • Collagen, type I, alpha 1-platelet-derived growth factor beta fusion gene (COL1A1-PDGFB) detectable by means of reverse transcriptase-polymerase chain reaction or FISH
  • Immunohistochemistry
    • + CD34

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Fibrosarcoma
  • MFH
  • Bednar tumor (pigmented [melanin] dermatofibrosarcoma protuberans, storiform neurofibroma)
  • Neurofibroma (+ S-100)
  • Myxoid liposarcoma
  • BFH (a spectrum between DFSP and BFH has been suggested)
  • Diffuse neurofibroma

DISEASE COURSE AND TREATMENT

  • Wide resection
  • Infrequently metastasizes (<5%) but to the lungs and LNs has been reported
  • Dedifferentiation to MFH or fibrosarcoma has been reported
  • Locally recurrences dependant upon extent of the wide excision
  • Wide excision of the tumor bed indicated when a marginal excision previously performed
  • LR after 23 yrshas been reported
    • The initial tumor was fibrosarcomatous DFSP and the recurrence was reported as a Bednar tumor

SPECIAL CONSIDERATIONS

  • BEDNAR TUMOR

 

    • Dermal and subcutaneous locally recurring painless lesion similar to DFSP ocurring on the trunk of (usually) children (juvenile form of DFSP)
    • M>F
    • Usually 1-8 cm in size
    • Cut surface is tan/brown ± focal hemorrhage and cyst formation
    • Well circumscribed with thick pseudocapsule
    • Spindle-shaped cells with cytoplasmic processes in a myxoid matrix
    • Multinucleated giant cells with bland "floret" nuclei
    • Cleft-like spaces
    • < 1 mitotic figure/10 hpf
    • Immunohistochemistry: + CD34
    • Has the same translocation as DFSP
    • DDx
      • Nodular fasciitis
      • Proliferative fasciitis
      • Proliferative myositis
      • Inflammatory myofibroblastic tumor
      • Benign fibrous histiocytoma
  • FIBROSARCOMATOUS OR MFH CHANGE WITHIN DFSP

 

REFERENCES

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