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Dermatofibrosarcoma protuberans ([[DFSP]]) is a [[rare]] non-[[hereditary]] [[neoplasm]] of the [[dermis]] layer of the [[skin]] which is sometimes described as having the tentacles [[Growth|growing]] into the surrounding [[fat]], [[muscle]] and even [[bone]] and is therefore, [[Classification|classified]] as a [[soft tissue]] [[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 a [[malignant]] [[potential]]. 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]]. | |||
Revision as of 15:21, 16 August 2019
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]
Dermatofibrosarcoma protuberans (DFSP) is a rare non-hereditary neoplasm of the dermis layer of the skin which is sometimes described as having the tentacles growing into the surrounding fat, muscle and even bone and is therefore, classified as a soft tissue sarcoma. In many respects, the disease behaves as a benign tumor, but in 2-5% of cases it can metastasize, so it should be considered to have a malignant potential. Over 95% of DFSP tumors have the chromosomal translocation t(17;22). The translocation fuses the collagen gene (COL1A1) with the platelet-derived growth factor gene. The fibroblast, the cell of origin of this tumor, expresses the fusion gene in the belief that it is collagen. However, the resulting fusion protein is processed into mature platelet-derived growth factor which is a potent growth factor. Fibroblasts contain the receptor for this growth factor. Thus, the cell "thinks" it is producing a structural protein, but in fact produces a self-stimulatory growth signal. The 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 body.