Dermatofibrosarcoma protuberans

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
Prognosis	moderate, locally aggressive, rarely metastases

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:

Dermatofibroma - main DDx - has entrapment of collagen bundles at the edge of the lesion.
Dermatomyofibroma.^[5]
Nodular fasciitis.

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:

DFSP (webpathology.com).

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.

References

↑ 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.
↑ ^2.0 ^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.
↑ ^3.0 ^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.
↑ 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.
↑ Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 504. ISBN 978-0443066542.
↑ AP. May 2009.
↑ ^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.
↑ ^8.0 ^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.
↑ 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.

[pmid21128251-1] 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.

[pmid23327727-2] 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.

[Ref_PBoD8_1183-3] 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.

[Ref_PCPBoD8_600-4] 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.

[Ref_Derm504-5] 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.

[pmid7694515-7] 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.

[pmid9129699-8] 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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Dermatofibrosarcoma protuberans

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