Difference between revisions of "Dermatofibrosarcoma protuberans"

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'''Dermatofibrosarcoma_protuberans''', abbreviated ''DFSP'', is a rare locally aggressive [[dermatologic neoplasms|tumour of the skin]].
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Storiform_pattern_-_very_high_mag.jpg
| Width      =
| Caption    = DFSP. [[H&E stain]].
| Micro      = dermal spindle cell lesion with storiform pattern, typically contains adipose tissue within the tumour -- described as "honeycomb pattern" and "Swiss cheese pattern"
| Subtypes  =
| LMDDx      = [[dermatofibroma]], [[dermatomyofibroma]], [[nodular fasciitis]]
| Stains    =
| IHC        = CD34 +ve, Factor XIIIa -ve
| EM        =
| Molecular  = t(17;22)(q22;q15)
| IF        =
| Gross      = firm plaque +/-ulceration
| Grossing  =
| Site      = [[skin]] - usually trunk
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence =
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
}}
'''Dermatofibrosarcoma protuberans''', abbreviated '''DFSP''', is a rare locally aggressive [[dermatologic neoplasms|tumour of the skin]].


==General==
==General==
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Notes:
Notes:
*Adnexal structure within tumour are preserved -- this is unusual for a malignant tumour -- '''important'''.
*Adnexal structure within tumour are preserved -- this is unusual for a malignant tumour -- '''important'''.


DDx:
DDx:
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*Factor XIIIa -ve.
*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>
**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).
*S-100 -ve (screen for melanoma).
*Caldesmin -ve (screen for muscle differentiation).
*Caldesmin -ve (screen for muscle differentiation).
*Beta-catenin. (???)
*Beta-catenin. (???)
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==See also==
==See also==
*[[Dermatologic neoplasms]].
*[[Dermatologic neoplasms]].
*[[Dermatofibroma]].


==References==
==References==
48,830

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