Difference between revisions of "Dermatologic neoplasms"

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Dermatologic neoplasms (view source)

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→‎Dermatofibrosarcoma protuberans: split-out
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==Dermatofibrosarcoma protuberans==
==Dermatofibrosarcoma protuberans==
*Abbreviated ''DFSP''.
*Abbreviated ''DFSP''.
===General===
{{Main|Dermatofibrosarcoma protuberans}}
*Dermal location.
*Destroys adnexal structures.
*Occasionally transforms to a (more aggressive) [[adult fibrosarcoma|fibrosarcoma]].<ref name=pmid21128251>{{Cite journal  | last1 = Stacchiotti | first1 = S. | last2 = Pedeutour | first2 = F. | last3 = Negri | first3 = T. | last4 = Conca | first4 = E. | last5 = Marrari | first5 = A. | last6 = Palassini | first6 = E. | last7 = Collini | first7 = P. | last8 = Keslair | first8 = F. | last9 = Morosi | first9 = C. | title = Dermatofibrosarcoma protuberans-derived fibrosarcoma: clinical history, biological profile and sensitivity to imatinib. | journal = Int J Cancer | volume = 129 | issue = 7 | pages = 1761-72 | month = Oct | year = 2011 | doi = 10.1002/ijc.25826 | PMID = 21128251 }}</ref>
 
Treatment:<ref name=Ref_PBoD8_1183>{{Ref PBoD8|1183}}</ref>
*Wide excision.
*May include [[imatinib]] (Gleevec).
 
===Gross===
Features:<ref name=Ref_PCPBoD8_600>{{Ref PCPBoD8|600}}</ref>
*Firm plaque, often bosselated, usually on the trunk.
*+/-Ulceration.
 
Images:
*[http://dermatlas.med.jhmi.edu/derm/display.cfm?ImageID=-375107780 Protuberant DFSP (dermatlas.med.jhmi.edu)].
*[http://dermatlas.med.jhmi.edu/derm/indexDisplay.cfm?ImageID=-1421097348 Huge DFSP on back (dermatlas.med.jhmi.edu)].
*[http://dermatlas.med.jhmi.edu/derm/indexDisplay.cfm?ImageID=-109598044 Protuberant DFSP - gross and histology (dermatlas.med.jhmi.edu)].
 
===Microscopic===
Features:<ref name=Ref_PBoD8_1183>{{Ref PBoD8|1183}}</ref>
*Dermal spindle cell lesion with storiform pattern.
**Spokes of the wheel-pattern.
*Contains adipose tissue within the tumour -- '''key feature'''.
**Described as "honeycomb pattern" and "Swiss cheese pattern".
 
Notes:
*Adnexal structure within tumour are preserved -- this is unusual for a malignant tumour -- '''important'''.
 
 
DDx:
*[[Dermatofibroma]] - main DDx - has entrapment of collagen bundles at the edge of the lesion.
*[[Dermatomyofibroma]].<ref name=Ref_Derm504>{{Ref Derm|504}}</ref>
*[[Nodular fasciitis]].
 
DDx of storiform pattern:
*DFSP.
*Dermatofibroma.
*[[Solitary fibrous tumour]].
*[[Undifferentiated pleomorphic sarcoma]].
 
====Images====
<gallery>
Image:SkinTumors-P9280838.JPG | DFSP with fat entrapped. (WC)
Image:SkinTumors-P9270829.JPG | DFSP - high mag. (WC)
Image:Storiform_pattern_-_intermed_mag.jpg | DFSP - storiform pattern - intermed. mag. (WC/Nephron)
Image:Storiform_pattern_-_very_high_mag.jpg | DFSP - storiform pattern - very high mag. (WC/Nephron)
</gallery>
www:
*[http://webpathology.com/image.asp?case=317&n=1 DFSP (webpathology.com)].
 
===IHC===
Panel:<ref>AP. May 2009.</ref>
*CD34 +ve.
**Usually negative in dermatofibroma.<ref name=pmid7694515>{{cite journal |author=Abenoza P, Lillemoe T |title=CD34 and factor XIIIa in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans |journal=Am J Dermatopathol |volume=15 |issue=5 |pages=429–34 |year=1993 |month=October |pmid=7694515 |doi= |url=}}</ref><ref name=pmid9129699>{{cite journal |author=Goldblum JR, Tuthill RJ |title=CD34 and factor-XIIIa immunoreactivity in dermatofibrosarcoma protuberans and dermatofibroma |journal=Am J Dermatopathol |volume=19 |issue=2 |pages=147–53 |year=1997 |month=April |pmid=9129699 |doi= |url=}}</ref>
*Factor XIIIa -ve.
**Usually positive in dermatofibroma.<ref name=pmid7694515>{{cite journal |author=Abenoza P, Lillemoe T |title=CD34 and factor XIIIa in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans |journal=Am J Dermatopathol |volume=15 |issue=5 |pages=429–34 |year=1993 |month=October |pmid=7694515 |doi= |url=}}</ref><ref name=pmid9129699>{{cite journal |author=Goldblum JR, Tuthill RJ |title=CD34 and factor-XIIIa immunoreactivity in dermatofibrosarcoma protuberans and dermatofibroma |journal=Am J Dermatopathol |volume=19 |issue=2 |pages=147–53 |year=1997 |month=April |pmid=9129699 |doi= |url=}}</ref>
*S100 -ve (screen for melanoma).
*Caldesmin -ve (screen for muscle differentiation).
*Beta-catenin. (???)
*MIB1 (proliferation marker).
**Should not be confused with ''MIB-1'' a gene that regulates [[apoptosis]].
 
===Molecular===
A characteristic [[translocation]] is seen:<ref>{{Ref PBoD8|1249}}</ref>
t(17;22)(q22;q15) COLA1/PDGFB.


==Cutaneous B-cell lymphoma==
==Cutaneous B-cell lymphoma==
Michael
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