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Dermatofibrosarcoma protuberans (view source)
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'''Dermatofibrosarcoma_protuberans''', abbreviated ''DFSP'', is a rare locally aggressive [[dermatologic neoplasms|tumour of the skin]]. | |||
==General== | |||
*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. | |||
==See also== | |||
*[[Dermatologic neoplasms]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Dermatologic neoplasms]] | |||