Difference between revisions of "Dermatofibrosarcoma protuberans"
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''' | {{ 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> | ||
* | *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== | ||