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| {{CMG}}; {{AE}} {{S.M.}} | | {{CMG}}; {{AE}} {{S.M.}} |
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| [[Dermatofibrosarcoma protuberans]] ([[DFSP]]) is a [[rare]] [[neoplasm]] of the [[dermis]] layer of the [[skin]], and is [[Classification|classified]] as a [[sarcoma]]. In many respects, the [[disease]] [[Behavior|behaves]] as a [[benign]] [[tumor]], but in 2-5% of [[Case-based reasoning|cases]] it can [[Metastasis|metastasize]], so it should be considered to have [[malignant]] [[potential]].
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| Over 95% of [[DFSP]] [[tumors]] have the [[chromosomal translocation]] t(17;22). The [[Translocations|translocation]] [[Fusion gene|fuses]] the [[collagen]] [[gene]] ([[COL1A1]]) with the [[PDGF|platelet-derived growth factor]] [[gene]]. The [[fibroblast]], the [[Cell (biology)|cell]] of [[origin]] of this [[tumor]], [[Expression|expresses]] the [[fusion gene]] in the belief that it is [[collagen]]. However, the [[Result|resulting]] [[fusion protein]] is [[Process (anatomy)|processed]] into mature [[PDGF|platelet-derived growth factor]] which is a [[Potential|potent]] [[growth factor]]. [[Fibroblasts]] contain the [[Receptor (biochemistry)|receptor]] for this [[growth factor]]. Thus, the [[Cell (biology)|cell]] "thinks" it is [[Product (biology)|producing]] a [[Structure factor|structural]] [[protein]], but in fact [[Product (biology)|produces]] a self-[[Stimulated emission|stimulatory]] [[growth]] [[Signal (biology)|signal]]. The [[Cell division|cell divides]] rapidly and a [[tumor]] forms. In [[dermatofibrosarcoma protuberans]], the [[tumor]] has a tendency to return after being removed. However, it does not often [[metastasize]] to other parts of the [[Human body|body]].
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Revision as of 22:28, 15 August 2019