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ToolsLim, Su X., Ramaiya, Arjun, Levell, Nick J. and Venables, Zoe C. (2023) Review of dermatofibrosarcoma protuberans. Clinical and Experimental Dermatology, 48 (4). pp. 297-302. ISSN 0307-6938
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The clinical features, histological subtypes and management of dermatofibrosarcoma protuberans (DFSP) are reviewed in this article. DFSP is an uncommon cutaneous sarcoma first described in 1890. It has a high local recurrence rate, low metastatic rate and low mortality. The crude incidence rate in England in 2019 was reported as 3.0 per million person-years. A fusion of platelet-derived growth factor subunit B (PDGFB) and COL1A1, t(17;22)(q22;q13), has been found in over 90% of people with DFSP. This fusion is thought to upregulate PDGFB expression, stimulating cell growth by activation of Ras mitogen-activated protein kinases and PI3K–AKT–mTOR, potentiating oncogenesis. DFSP usually presents as an asymptomatic flesh-coloured, thickened, rubbery plaque or nodule with an uneven surface. The most common sites are the trunk followed by lower limbs, head and neck and upper limbs. Larger tumours can infiltrate underlying local structures and around 1% metastasize. Key histological features in DFSP are spindle cells arranged in a storiform pattern with intense CD34 staining. Histological subtypes include classical DFSP, Bednar, myxoid, giant cell fibroblastoma, atrophic and DFSP–fibrosarcomatous. The gold standard management for localized tumours is surgical: current recommendations favour Mohs micrographic surgery over wide local excision. Adjuvant radiotherapy may be offered after surgery. Imatinib can be used as neoadjuvant therapy and in patients with inoperable or metastatic tumours. Further research should be conducted to better understand pathogenesis of DFSP, identify associated risk factors and standardize management.
| Item Type: | Article |
|---|---|
| Additional Information: | Publisher Copyright: © The Author(s) 2022. Published by Oxford University Press on behalf of British Association of Dermatologists. All rights reserved. |
| Uncontrolled Keywords: | dermatology ,/dk/atira/pure/subjectarea/asjc/2700/2708 |
| Faculty \ School: | Faculty of Medicine and Health Sciences > Norwich Medical School |
| UEA Research Groups: | Faculty of Medicine and Health Sciences > Research Centres > Population Health Faculty of Science > Research Groups > Norwich Epidemiology Centre Faculty of Medicine and Health Sciences > Research Groups > Norwich Epidemiology Centre |
| Related URLs: | |
| Depositing User: | LivePure Connector |
| Date Deposited: | 31 Mar 2025 15:30 |
| Last Modified: | 31 Mar 2025 15:30 |
| URI: | https://ueaeprints.uea.ac.uk/id/eprint/98911 |
| DOI: | 10.1093/ced/llac111 |
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