Dermatofibrosarcoma protuberans (DFSP) is a hypercellular, storiform, CD34+ low-grade sarcoma with honeycomb entrapment of fat, which typically involves the trunk and extremities.
Differential diagnosis:
dermatofibroma (fibrous histiocytoma)(CD34, Factor XIIIa, CD44, hyaluronate) (12641778)
Variants
occurence in a smallpox vaccination scar (12734474)
congenital dermatofibrosarcoma protuberans (12588227)
childhood-onset dermatofibrosarcoma protuberans
pigmented dermatofibrosarcoma protuberans (Bednar tumor)
granular cell variant of dermatofibrosarcoma protuberans (12131162)
atrophic dermatofibrosarcoma
multifocal dermatofibrosarcoma
superficial adult fibrosarcoma with COL1A1/PDGFB fusion transcript expression
myxoid dermatofibrosarcoma protuberans (17721193)
in two brothers (17637382)
Cytogenetics
translocation t(17;22)(q22;q13) creating COL1A1/PDGFB fusion gene
translocation t(9;22)(q32;q12.2)
translocation t(2;17)
Molecular biology
COL1A1/PDGFB fusion protein by t(17;22)(q22;q13) (COL1A1 at 17q22 and PDGFB at 22q13
Localization
cutaneous DFSP
oral DFSP (10534672)
labial DFSP (9236907)
jejeunal DFSP (11206873)
Spradic ssociations
nuchal-type fibroma (14675287)
References
Takahira T, Oda Y, Tamiya S, Yamamoto H, Kawaguchi K, Kobayashi C, Oda S, Iwamoto Y, Tsuneyoshi M. Microsatellite instability and p53 mutation associated with tumor progression in dermatofibrosarcoma protuberans. Hum Pathol. 2004 Feb;35(2):240-5. PMID: 14991543
Kahn HJ, Fekete E, From L. Tenascin differentiates dermatofibroma from dermatofibrosarcoma protuberans: comparison with CD34 and factor XIIIa. Hum Pathol. 2001 Jan;32(1):50-6. PMID: 11172295