Dermatofibrosarcoma protuberans: Difference between revisions

Jump to navigation Jump to search
← Older edit
Michael (talk | contribs)
49,345 edits
split-out
Michael (talk | contribs)
49,345 edits
tweak
 
(5 intermediate revisions by the same user not shown)
Line 1: Line 1:
'''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 or proximal extremities
| Assdx      =
| Syndromes  =
| Clinicalhx = second to fifth decade
| Signs      =
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = moderate, locally aggressive, rarely metastases
| Other      =
| ClinDDx    =
| Tx = wide excision
}}
'''Dermatofibrosarcoma protuberans''', abbreviated '''DFSP''', is a rare locally aggressive [[dermatologic neoplasms|tumour of the skin]].


==General==
==General==
*Dermal location.
*Destroys adnexal structures - somewhat unusual for a mostly benign tumour.
*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>
*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>
*Typically slow growing.<ref name=pmid23327727>{{Cite journal  | last1 = Llombart | first1 = B. | last2 = Serra-Guillén | first2 = C. | last3 = Monteagudo | first3 = C. | last4 = López Guerrero | first4 = JA. | last5 = Sanmartín | first5 = O. | title = Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management. | journal = Semin Diagn Pathol | volume = 30 | issue = 1 | pages = 13-28 | month = Feb | year = 2013 | doi = 10.1053/j.semdp.2012.01.002 | PMID = 23327727 }}</ref>
*Usually second to fifth decade.<ref name=pmid23327727/>


Treatment:<ref name=Ref_PBoD8_1183>{{Ref PBoD8|1183}}</ref>
Treatment:<ref name=Ref_PBoD8_1183>{{Ref PBoD8|1183}}</ref>
Line 29: Line 60:
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:
Line 41: Line 71:
*[[Solitary fibrous tumour]].
*[[Solitary fibrous tumour]].
*[[Undifferentiated pleomorphic sarcoma]].
*[[Undifferentiated pleomorphic sarcoma]].
===Subtypes===
Numerous variants/subtypes are described:<ref name=pmid23327727>{{Cite journal  | last1 = Llombart | first1 = B. | last2 = Serra-Guillén | first2 = C. | last3 = Monteagudo | first3 = C. | last4 = López Guerrero | first4 = JA. | last5 = Sanmartín | first5 = O. | title = Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management. | journal = Semin Diagn Pathol | volume = 30 | issue = 1 | pages = 13-28 | month = Feb | year = 2013 | doi = 10.1053/j.semdp.2012.01.002 | PMID = 23327727 }}</ref>
*Pigmented DFSP (Bednar tumour).
*Myxoid DFSP.
*Myoid DFSP.
*Granular cell DFSP.
*Sclerotic DFSP.
*Atrophic DFSP,
*Giant cell fibroblastoma.
*DFSP with fibrosarcomatous areas.


===Images===
===Images===
Line 58: Line 99:
*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. (???)
Line 70: Line 111:
==See also==
==See also==
*[[Dermatologic neoplasms]].
*[[Dermatologic neoplasms]].
*[[Dermatofibroma]].


==References==
==References==
Retrieved from "https://librepathology.org/wiki/Dermatofibrosarcoma_protuberans"