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{{CMG}}; {{AE}} {{S.M.}}
{{CMG}}; {{AE}} {{S.M.}}


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]].
* Dermatofibrosarcoma protuberans is [[Estimate|estimated]] to occur in 1 in 100,000 to 1-5 in 1 million people per [[year]]
* It usually occurs in [[Adult|adults]] of 20 - 40 [[Year|years]] of [[age]]
* It affects twice more commonly the [[Black|blacks]] than whites in [[United States|US]]<ref name="pmid17141362">{{cite journal| author=Criscione VD, Weinstock MA| title=Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002. | journal=J Am Acad Dermatol | year= 2007 | volume= 56 | issue= 6 | pages= 968-73 | pmid=17141362 | doi=10.1016/j.jaad.2006.09.006 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17141362  }} </ref>
* It can also occur in [[infants]] and [[children]]<ref name="pmid18096453">{{cite journal| author=Reddy C, Hayward P, Thompson P, Kan A| title=Dermatofibrosarcoma protuberans in children. | journal=J Plast Reconstr Aesthet Surg | year= 2009 | volume= 62 | issue= 6 | pages= 819-23 | pmid=18096453 | doi=10.1016/j.bjps.2007.11.009 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18096453  }} </ref><ref name="pmid20952167">{{cite journal| author=Zaraa I, Ben abdallah M, Driss M, Trojjet S, Ben Sassi M, El Euch D et al.| title=[Dermatofibrosarcoma protuberans in children]. | journal=Arch Pediatr | year= 2011 | volume= 18 | issue= 1 | pages= 23-7 | pmid=20952167 | doi=10.1016/j.arcped.2010.09.010 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20952167  }} </ref><ref name="pmid17310000">{{cite journal| author=Maire G, Fraitag S, Galmiche L, Keslair F, Ebran N, Terrier-Lacombe MJ et al.| title=A clinical, histologic, and molecular study of 9 cases of congenital dermatofibrosarcoma protuberans. | journal=Arch Dermatol | year= 2007 | volume= 143 | issue= 2 | pages= 203-10 | pmid=17310000 | doi=10.1001/archderm.143.2.203 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17310000  }} </ref>
* It has a [[Stable element|stable]] [[incidence]] which is highest among [[Womens Pack|women]]<ref name="pmid26730971">{{cite journal| author=Kreicher KL, Kurlander DE, Gittleman HR, Barnholtz-Sloan JS, Bordeaux JS| title=Incidence and Survival of Primary Dermatofibrosarcoma Protuberans in the United States. | journal=Dermatol Surg | year= 2016 | volume= 42 Suppl 1 | issue=  | pages= S24-31 | pmid=26730971 | doi=10.1097/DSS.0000000000000300 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26730971  }} </ref><ref name="pmid22691126">{{cite journal| author=Kuzel P, Metelitsa AI, Dover DC, Salopek TG| title=Epidemiology of dermatofibrosarcoma protuberans in Alberta, Canada, from 1988 to 2007. | journal=Dermatol Surg | year= 2012 | volume= 38 | issue= 9 | pages= 1461-8 | pmid=22691126 | doi=10.1111/j.1524-4725.2012.02482.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22691126  }} </ref>
* Worse [[Survival analysis|survival]] is [[Association (statistics)|associated]] with:
** Increased [[age]]
**[[Male]] [[Sex (activity)|sex]]
**[[Black]] [[race]]
**[[Anatomic]] [[Location parameter|location]] of the [[limbs]] and [[head]]

Revision as of 15:24, 16 August 2019


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]

  1. ↑ Criscione VD, Weinstock MA (2007). "Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002". J Am Acad Dermatol. 56 (6): 968–73. doi:10.1016/j.jaad.2006.09.006. PMID 17141362.
  2. ↑ Reddy C, Hayward P, Thompson P, Kan A (2009). "Dermatofibrosarcoma protuberans in children". J Plast Reconstr Aesthet Surg. 62 (6): 819–23. doi:10.1016/j.bjps.2007.11.009. PMID 18096453.
  3. ↑ Zaraa I, Ben abdallah M, Driss M, Trojjet S, Ben Sassi M, El Euch D; et al. (2011). "[Dermatofibrosarcoma protuberans in children]". Arch Pediatr. 18 (1): 23–7. doi:10.1016/j.arcped.2010.09.010. PMID 20952167.
  4. ↑ Maire G, Fraitag S, Galmiche L, Keslair F, Ebran N, Terrier-Lacombe MJ; et al. (2007). "A clinical, histologic, and molecular study of 9 cases of congenital dermatofibrosarcoma protuberans". Arch Dermatol. 143 (2): 203–10. doi:10.1001/archderm.143.2.203. PMID 17310000.
  5. ↑ Kreicher KL, Kurlander DE, Gittleman HR, Barnholtz-Sloan JS, Bordeaux JS (2016). "Incidence and Survival of Primary Dermatofibrosarcoma Protuberans in the United States". Dermatol Surg. 42 Suppl 1: S24–31. doi:10.1097/DSS.0000000000000300. PMID 26730971.
  6. ↑ Kuzel P, Metelitsa AI, Dover DC, Salopek TG (2012). "Epidemiology of dermatofibrosarcoma protuberans in Alberta, Canada, from 1988 to 2007". Dermatol Surg. 38 (9): 1461–8. doi:10.1111/j.1524-4725.2012.02482.x. PMID 22691126.
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