Dermatofibrosarcoma Protruberans A Locally Aggressive And Recurent Malignant Tumour Of The Skin: Review And Case Report

  • Malumani Malan Zhongnan Hospital of Wuhan University
  • Song Ji Quan Zhongnan Hospital of Wuhan University
  • Wu Jianbo Zhongnan Hospital of Wuhan University
  • Jiang Si Zhongnan Hospital of Wuhan University
  • Musa Gerald Livingstone Central Hospital
Keywords: dermatofibroma, dermatofibrosarcoma protruberans, Lipoma, fibrosarcomatous, recurrence, aggressive cutaneous tumour

Abstract

Dermatofibrosarcoma protuberans (DFSP) is uncommon but represents one of the most common dermal sarcomas, which is said to be a locally aggressive, low grade, and relatively uncommon cutaneous tumour, which has a high propensity for local relapse with low metastatic potential. Over 90% DFSPs are genetically characterized by chromosomal rearrangements (translocation t(17;22) (q22; q13), resulting in the collagen type-1 alpha 1 (COL1A1)-platelet-derived growth factor â (PDGFB) fusion gene with well-known risk factors for developing DFSP, some cases develop at the site of previous trauma and reports have included a burn scar and the site of vaccination. The mainstay mode of treatment of DFSPs is wide local excision with traditionally 3-cm gross margins excision surgery. Mohs Micrographic Surgery (MMS) is highly recommended surgery for DFSP and is very useful treatment choice for recurrent DFSP. This case report discusses two patients with confirmed DFSP and have received adequate therapy. It also reviews salient features of evaluation and prompt management of DFSP and much emphasis on long-term follow-up of patients. Key Messages: Early diagnosis of pediatric DFSP is critical to minimize surgical disfiguration. We emphasize early evaluation of any suspicious skin lesion should not be ignored and should be excised and sent for histopathology. Undertreating a DFSP is even more problematic, as the tumour is locally destructive, can metastasize, and can prove fatal without treatment. A full metastatic workup should be done and patient should be regularly followed up, if it turns out to be malignant.

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Author Biographies

Malumani Malan, Zhongnan Hospital of Wuhan University

Department of Dermatology and Venereology

Song Ji Quan, Zhongnan Hospital of Wuhan University

Head of Department of Dermatology and Venereology

Wu Jianbo, Zhongnan Hospital of Wuhan University

Department of Dermatology and Venereology

Jiang Si, Zhongnan Hospital of Wuhan University

Department of Dermatology and Venereology

Musa Gerald, Livingstone Central Hospital

Department of Surgery

Published
2018-07-11
Section
Articles