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


[[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]].


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]].
 
<ref name="pmid27261907">{{cite journal| author=Mao Y, Yang D, He J, Krasna MJ| title=Epidemiology of Lung Cancer. | journal=Surg Oncol Clin N Am | year= 2016 | volume= 25 | issue= 3 | pages= 439-45 | pmid=27261907 | doi=10.1016/j.soc.2016.02.001 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27261907  }} </ref>
 
[[File:Atypical Bcell gif.gif|thumb|200px|none|High-power field of peripheral blood smear revealing a large, atypical B cell with mild cytoplasmic expansion, coarse chromatin, multiple distinct nucleoli and peripheral vacuolation.[https://openi.nlm.nih.gov/detailedresult.php?img=PMC2944189_1752-1947-4-300-2&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=17 Source: Charakidis M. et al, Department of Haematology-Oncology, Royal Hobart Hospital, Tasmania, 7000, Australia.]]]
 
 
{|
|+'''Classification of Waldenstrom macroglobulinemia (WM) and Related Disorders'''
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Criteria
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Symptomatic WM
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Asymptomatic WM
! style="background:#4479BA; color: #FFFFFF;" align="center" + |IgM-Related Disorders
! style="background:#4479BA; color: #FFFFFF;" align="center" + |MGUS
|-
! align="center" style="background:#DCDCDC;" + |IgM monoclonal protein
| style="background:#F5F5F5;" align="center" + | +
| style="background:#F5F5F5;" align="center" + | +
| style="background:#F5F5F5;" align="center" + | +
| style="background:#F5F5F5;" align="center" + | +
|-
! align="center" style="background:#DCDCDC;" + |Bone marrow infiltration
| style="background:#F5F5F5;" align="center" + | +
| style="background:#F5F5F5;" align="center" + | +
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + | -
|-
! align="center" style="background:#DCDCDC;" + |Symptoms attributable to IgM
| style="background:#F5F5F5;" align="center" + | +
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + | +
| style="background:#F5F5F5;" align="center" + | -
|-
! align="center" style="background:#DCDCDC;" + |Symptoms attributable to tumor infiltration
| style="background:#F5F5F5;" align="center" + | +
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + | -
| style="background:#F5F5F5;" align="center" + | -
|}<br />
 
 
 
 
 
==Reference==
{{Reflist|2}}

Latest revision as of 18:18, 11 December 2020


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


[1]

High-power field of peripheral blood smear revealing a large, atypical B cell with mild cytoplasmic expansion, coarse chromatin, multiple distinct nucleoli and peripheral vacuolation.Source: Charakidis M. et al, Department of Haematology-Oncology, Royal Hobart Hospital, Tasmania, 7000, Australia.


Classification of Waldenstrom macroglobulinemia (WM) and Related Disorders
Criteria Symptomatic WM Asymptomatic WM IgM-Related Disorders MGUS
IgM monoclonal protein + + + +
Bone marrow infiltration + + - -
Symptoms attributable to IgM + - + -
Symptoms attributable to tumor infiltration + - - -




Reference

  1. ↑ Mao Y, Yang D, He J, Krasna MJ (2016). "Epidemiology of Lung Cancer". Surg Oncol Clin N Am. 25 (3): 439–45. doi:10.1016/j.soc.2016.02.001. PMID 27261907.
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