Dermatofibrosarcoma protuberans

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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

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.