Abstract
Dermatofibrosarcoma protuberans is a superficial malignancy (included among skin sarcomas). The cellular origin is unknown, potential cell candidates being histiocytes, fibroblasts, and dendritic cells (it is currently classified among fibroblastic-myofibroblastic soft tissue tumors).
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
Notes
- 1.
COL1A1-PDGFB fusion gene: COL1A1 (collagen type I alpha 1 chain) encodes the pro-alpha1 chains of type I collagen whose triple helix comprises two alpha1 chains and one alpha2 chain. Type I is a fibril-forming collagen found in most connective tissues and is abundant in the bone, cornea, dermis, and tendon. PDGFB encodes platelet-derived growth factor subunit B, a member of the protein family comprised of both platelet-derived growth factors (PDGF) and vascular endothelial growth factors (VEGF); the encoded preproprotein is proteolytically processed to generate platelet-derived growth factor subunit B, which can homodimerize, or, alternatively, heterodimerize with the related platelet-derived growth factor subunit A: ultimately, these proteins bind and activate PDGF receptor (PDGFR) tyrosine kinases, which play a role in a wide range of cell activities.
- 2.
RT-PCR: reverse transcriptase-polymerase chain reaction.
- 3.
FISH: fluorescence in situ hybridization.
- 4.
Imatinib: tyrosine kinase inhibitor targeting the following—KIT, RET, BCR, PDGFRA.
- 5.
Sunitinib: tyrosine kinase inhibitor targeting the following—PDGFR, VEGFR, KIT, FLT3, CSF1R, RET.
- 6.
PDGFR: platelet-derived growth factor receptor is a family of tyrosine kinase receptors including PDGFR alpha (PDGFRA) and beta (PDGFRB). The receptor is activated by binding to its ligands, which belong to the PDGF family, PDGFA, PDGFB, PDGFC, and PDGFD, which form either homodimers or heterodimers (PDGF-AA, -AB, -BB, -CC, -DD). Depending on which growth factor is bound, PDGFR homo- or heterodimerizes. These growth factors are mitogens for cells of mesenchymal origin and play an important role in organ development, wound healing, and tumor progression.
Suggested Readings
Allen (2019) Dermatofibrosarcoma protuberans. Dermatol Clin 37(4):483–488
Fletcher (2020) WHO classification of tumours of soft tissue and bone, 5th edn. IARC Press, Lyon
Hornick (2020) Cutaneous soft tissue tumors: how do we make sense of fibrous and “fibrohistiocytic” tumors with confusing names and similar appearances? Mod Pathol 33:56–65 [Epub ahead of print]
Iwasaki (2019) Current update on the molecular biology of cutaneous sarcoma: dermatofibrosarcoma protuberans. Curr Treat Options Oncol 20(4):29
Köster (2020) Genomic and transcriptomic features of dermatofibrosarcoma protuberans: Unusual chromosomal origin of the COL1A1-PDGFB fusion gene and synergistic effects of amplified regions in tumor development. Cancer Genet 241:34–41
Malhotra (2012) Dermatofibrosarcoma protruberans treatment with platelet-derived growth factor receptor inhibitor: a review of clinical trial results. Curr Opin Oncol 24(4):419–424
Navarrete-Dechent (2019) Imatinib treatment for locally advanced or metastatic dermatofibrosarcoma protuberans: a systematic review. JAMA Dermatol 155(3):361–369
Rouhani (2008) Cutaneous soft tissue sarcoma incidence patterns in the U.S.: an analysis of 12,114 cases. Cancer 113(3):616–627
Snow (2020) Conservative re-excision is a safe and simple alternative to radical resection in revision surgery for dermatofibrosarcoma protuberans. Ann Surg Oncol 27(3):919–923
Ugurel (2014) Neoadjuvant imatinib in advanced primary or locally recurrent dermatofibrosarcoma protuberans: a multicenter phase II DeCOG trial with long-term follow-up. Clin Cancer Res 20(2):499–510
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
Copyright information
© 2021 The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG
About this chapter
Cite this chapter
Mocellin, S. (2021). Dermatofibrosarcoma Protuberans. In: Soft Tissue Tumors . Springer, Cham. https://doi.org/10.1007/978-3-030-58710-9_67
Download citation
DOI: https://doi.org/10.1007/978-3-030-58710-9_67
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-58709-3
Online ISBN: 978-3-030-58710-9
eBook Packages: MedicineMedicine (R0)