DEFINITION AND PATHOGENESIS
- A nodular cutaneous low-grade infiltrative malignant tumor with a high propensity for local recurrences
- Should be recognized for its aggressiveness to avoid recurrences and potential metastases
- 6% of all soft tissue sarcomas
- Nodular cutaneous mass
- Early or mid-adult life
- 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
- 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
- Solitary protuberant, grayish, infiltrative mass involving skin and/or subcutaneous tissue
- 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
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- 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
- Dermal and subcutaneous locally recurring painless lesion similar to DFSP ocurring on the trunk of (usually) children (juvenile form of DFSP)
- 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
- Nodular fasciitis
- Proliferative fasciitis
- Proliferative myositis
- Inflammatory myofibroblastic tumor
- Benign fibrous histiocytoma
FIBROSARCOMATOUS OR MFH CHANGE WITHIN DFSP
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