Difference between revisions of "Dermatofibrosarcoma protuberans"

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#redirect [[Dermatofibrosarcoma protuberans]]
{{ 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==
*Destroys adnexal structures - somewhat unusual for a mostly benign tumour.
*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>
*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]].
 
===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===
<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>
*S-100 -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]].
*[[Dermatofibroma]].
 
==References==
{{Reflist|2}}
 
[[Category:Diagnosis]]
[[Category:Dermatologic neoplasms]]

Latest revision as of 11:35, 21 June 2014

Dermatofibrosarcoma protuberans
Diagnosis in short

DFSP. H&E stain.
LM dermal spindle cell lesion with storiform pattern, typically contains adipose tissue within the tumour -- described as "honeycomb pattern" and "Swiss cheese pattern"
LM DDx dermatofibroma, dermatomyofibroma, nodular fasciitis
IHC CD34 +ve, Factor XIIIa -ve
Molecular t(17;22)(q22;q15)
Gross firm plaque +/-ulceration
Site skin - usually trunk or proximal extremities
Clinical history second to fifth decade
Prevalence uncommon
Prognosis moderate, locally aggressive, rarely metastases
Treatment wide excision

Dermatofibrosarcoma protuberans, abbreviated DFSP, is a rare locally aggressive tumour of the skin.

General

  • Destroys adnexal structures - somewhat unusual for a mostly benign tumour.
  • Occasionally transforms to a (more aggressive) fibrosarcoma.[1]
  • Typically slow growing.[2]
  • Usually second to fifth decade.[2]

Treatment:[3]

  • Wide excision.
  • May include imatinib (Gleevec).

Gross

Features:[4]

  • Firm plaque, often bosselated, usually on the trunk.
  • +/-Ulceration.

Images:

Microscopic

Features:[3]

  • 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:

DDx of storiform pattern:

Subtypes

Numerous variants/subtypes are described:[2]

  • Pigmented DFSP (Bednar tumour).
  • Myxoid DFSP.
  • Myoid DFSP.
  • Granular cell DFSP.
  • Sclerotic DFSP.
  • Atrophic DFSP,
  • Giant cell fibroblastoma.
  • DFSP with fibrosarcomatous areas.

Images

  • DFSP with fat entrapped. (WC)

  • DFSP - high mag. (WC)

  • DFSP - storiform pattern - intermed. mag. (WC/Nephron)

  • DFSP - storiform pattern - very high mag. (WC/Nephron)

www:

IHC

Panel:[6]

  • CD34 +ve.
    • Usually negative in dermatofibroma.[7][8]
  • Factor XIIIa -ve.
    • Usually positive in dermatofibroma.[7][8]
  • S-100 -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:[9] t(17;22)(q22;q15) COLA1/PDGFB.

See also

References

  1. ↑ Stacchiotti, S.; Pedeutour, F.; Negri, T.; Conca, E.; Marrari, A.; Palassini, E.; Collini, P.; Keslair, F. et al. (Oct 2011). "Dermatofibrosarcoma protuberans-derived fibrosarcoma: clinical history, biological profile and sensitivity to imatinib.". Int J Cancer 129 (7): 1761-72. doi:10.1002/ijc.25826. PMID 21128251.
  2. ↑ 2.0 2.1 2.2 Llombart, B.; Serra-Guillén, C.; Monteagudo, C.; López Guerrero, JA.; Sanmartín, O. (Feb 2013). "Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management.". Semin Diagn Pathol 30 (1): 13-28. doi:10.1053/j.semdp.2012.01.002. PMID 23327727.
  3. ↑ 3.0 3.1 Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1183. ISBN 978-1416031215.
  4. ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 600. ISBN 978-1416054542.
  5. ↑ Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 504. ISBN 978-0443066542.
  6. ↑ AP. May 2009.
  7. ↑ 7.0 7.1 Abenoza P, Lillemoe T (October 1993). "CD34 and factor XIIIa in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans". Am J Dermatopathol 15 (5): 429–34. PMID 7694515.
  8. ↑ 8.0 8.1 Goldblum JR, Tuthill RJ (April 1997). "CD34 and factor-XIIIa immunoreactivity in dermatofibrosarcoma protuberans and dermatofibroma". Am J Dermatopathol 19 (2): 147–53. PMID 9129699.
  9. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1249. ISBN 978-1416031215.
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