Editing Soft tissue sarcoma (section)

=== '''Dermatofibrosarcoma protuberans''' ===

** Superficial tumour that infiltrates soft tissue for centimetres beyond the obvious margins of the lesion and can recur locally after inadequate resection
** Pathogenesis relates to chromosomal translocation leading to abnormal fusion protein COL1A1-PDGFB
** Usually involves dermis and subcutis
** Usually fairly low-grade, metastases uncommon
** Surgery - need negative margins. Frozen section doesn't work because the fat doesn't freeze well for analysis!
** Usually superficial tumour, so deep resection of muscle is not generally necessary
** Radiotherapy can be used for positive or close margins, or for unresectable recurrences
** Imatinib can be effective adjuvant therapy
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