Review of dermatofibrosarcoma protuberans

Tools

Lim, 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

Full text not available from this repository. (Request a copy)

Abstract

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: 06 Apr 2025 06:30
URI: https://ueaeprints.uea.ac.uk/id/eprint/98911
DOI: 10.1093/ced/llac111

Actions (login required)

View Item View Item