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


* 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>
<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>
* 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>
[[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.]]]
* Worse [[Survival analysis|survival]] is [[Association (statistics)|associated]] with:
 
** Increased [[age]]
 
**[[Male]] [[Sex (activity)|sex]]
{|  
**[[Black]] [[race]]
|+'''Classification of Waldenstrom macroglobulinemia (WM) and Related Disorders'''
**[[Anatomic]] [[Location parameter|location]] of the [[limbs]] and [[head]]
! 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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