Atrophic dermatofibrosarcoma protuberans: report of a case demonstrated by detecting COL1A1-PDGFB rearrangement
<p>Abstract</p> <p>Dermatofibrosarcoma protuberans is a locally aggressive mesenchymal neoplasm. It usually presents as an indurated plaque that protrudes above the surface of the skin. Some patients have clinically persistent plaques that might be atrophic. The atrophic variant of...
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doaj-ee046a70d7a74544810db0cfdc8e00602020-11-24T23:26:35ZengBMCDiagnostic Pathology1746-15962012-11-017116610.1186/1746-1596-7-166Atrophic dermatofibrosarcoma protuberans: report of a case demonstrated by detecting COL1A1-PDGFB rearrangementQiao JianjunPatel Kayuri ULópez-Terrada DoloresFang Hong<p>Abstract</p> <p>Dermatofibrosarcoma protuberans is a locally aggressive mesenchymal neoplasm. It usually presents as an indurated plaque that protrudes above the surface of the skin. Some patients have clinically persistent plaques that might be atrophic. The atrophic variant of dermatofibrosarcoma protuberans may be confused with some common skin diseases with atrophic appearance. We reported a 40-year-old woman who had a 10-year history of an atrophic dermatofibrosarcoma protuberans. Molecular analysis showed a fusion between <it>COL1A1</it> exon 31 to exon 2 of <it>PDGFB</it>. The lesion was totally excised, with negative margins of the resection demonstrated by CD34 immunostaining. To our knowledge, this is the second case of atrophic dermatofibrosarcoma protuberans confirmed by detection of COL1A1-PDGFB fusion gene. This appears to be the first report of a fusion between <it>COL1A1</it> exon 31 to exon 2 of <it>PDGFB</it> in atrophic dermatofibrosarcoma protuberans.</p> <p>Virtual slides</p> <p>The virtual slides for this article can be found here: <url>http://www.diagnosticpathology.diagnomx.eu/vs/1249657688795311</url></p> http://www.diagnosticpathology.org/content/7/1/166Dermatofibrosarcoma protuberansCOL1A1-PDGFBCD34AtrophicSarcoma |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Qiao Jianjun Patel Kayuri U López-Terrada Dolores Fang Hong |
spellingShingle |
Qiao Jianjun Patel Kayuri U López-Terrada Dolores Fang Hong Atrophic dermatofibrosarcoma protuberans: report of a case demonstrated by detecting COL1A1-PDGFB rearrangement Diagnostic Pathology Dermatofibrosarcoma protuberans COL1A1-PDGFB CD34 Atrophic Sarcoma |
author_facet |
Qiao Jianjun Patel Kayuri U López-Terrada Dolores Fang Hong |
author_sort |
Qiao Jianjun |
title |
Atrophic dermatofibrosarcoma protuberans: report of a case demonstrated by detecting COL1A1-PDGFB rearrangement |
title_short |
Atrophic dermatofibrosarcoma protuberans: report of a case demonstrated by detecting COL1A1-PDGFB rearrangement |
title_full |
Atrophic dermatofibrosarcoma protuberans: report of a case demonstrated by detecting COL1A1-PDGFB rearrangement |
title_fullStr |
Atrophic dermatofibrosarcoma protuberans: report of a case demonstrated by detecting COL1A1-PDGFB rearrangement |
title_full_unstemmed |
Atrophic dermatofibrosarcoma protuberans: report of a case demonstrated by detecting COL1A1-PDGFB rearrangement |
title_sort |
atrophic dermatofibrosarcoma protuberans: report of a case demonstrated by detecting col1a1-pdgfb rearrangement |
publisher |
BMC |
series |
Diagnostic Pathology |
issn |
1746-1596 |
publishDate |
2012-11-01 |
description |
<p>Abstract</p> <p>Dermatofibrosarcoma protuberans is a locally aggressive mesenchymal neoplasm. It usually presents as an indurated plaque that protrudes above the surface of the skin. Some patients have clinically persistent plaques that might be atrophic. The atrophic variant of dermatofibrosarcoma protuberans may be confused with some common skin diseases with atrophic appearance. We reported a 40-year-old woman who had a 10-year history of an atrophic dermatofibrosarcoma protuberans. Molecular analysis showed a fusion between <it>COL1A1</it> exon 31 to exon 2 of <it>PDGFB</it>. The lesion was totally excised, with negative margins of the resection demonstrated by CD34 immunostaining. To our knowledge, this is the second case of atrophic dermatofibrosarcoma protuberans confirmed by detection of COL1A1-PDGFB fusion gene. This appears to be the first report of a fusion between <it>COL1A1</it> exon 31 to exon 2 of <it>PDGFB</it> in atrophic dermatofibrosarcoma protuberans.</p> <p>Virtual slides</p> <p>The virtual slides for this article can be found here: <url>http://www.diagnosticpathology.diagnomx.eu/vs/1249657688795311</url></p> |
topic |
Dermatofibrosarcoma protuberans COL1A1-PDGFB CD34 Atrophic Sarcoma |
url |
http://www.diagnosticpathology.org/content/7/1/166 |
work_keys_str_mv |
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