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Published on: April 11, 2018
Establishment and characterization of a novel imatinib-resistant dermatofibrosarcoma protuberans cell line, DFSP-DPH1
Hao Jiang1, Kunqian He1, Danli Li1
1Department of Plastic and Cosmetic Surgery, Daping Hospital, Army Medical University, No.10 Changjiang Branch Street, Yuzhong District, Chongqing, 40042, China.
Abstract:
Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive cutaneous sarcoma characterized by high recurrence rates and the development of resistance to imatinib. The scarcity of preclinical models hinders research into DFSP pathogenesis and the development of novel therapeutic strategies. In this study, we established and characterized a novel DFSP cell line, designated DFSP-DPH1, derived from a 47-year-old male patient with an abdominal tumor. Comprehensive characterization confirmed that DFSP-DPH1 retains key features of the original tumor, including the fibroblast-like spindle morphology and expression of diagnostic markers CD34 and vimentin, with absence of factor XIIIa. Short tandem repeat profiling confirmed the cell line's origin and excluded cross-contamination. Sanger sequencing revealed a COL1A1 exon 46-PDGFB exon 2 fusion transcript, a breakpoint not previously reported in established DFSP cell lines. Functionally, DFSP-DPH1 exhibits robust proliferative capacity, forms three-dimensional spheroids under anchorage-independent conditions, and demonstrates significant migratory and invasive capabilities. Drug sensitivity screening of a panel of 48 PDGFR inhibitors confirmed its resistance to imatinib and identified several compounds with superior efficacy compared to imatinib. Transcriptomic analysis confirmed the dominance of the COL1A1::PDGFB fusion transcript and revealed enrichment of pathways related to cancer, viral infection, and neuroactive ligand-receptor interaction. This novel imatinib-resistant DFSP cell line, DFSP-DPH1 provides a valuable preclinical model for investigating the molecular mechanisms underlying DFSP pathogenesis, drug resistance, and tumor progression, and for developing and evaluating novel therapeutic strategies.
Insights
A new imatinib-resistant Dermatofibrosarcoma protuberans (DFSP) cell line, DFSP-DPH1, was developed. This model aids research into DFSP progression and novel therapies for this rare skin cancer.
Area of Science:
- Oncology
- Dermatology
- Cancer Biology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive skin cancer with high recurrence rates.
- Existing preclinical models are limited, hindering research into DFSP pathogenesis and treatment resistance, particularly to imatinib.
- The development of novel therapeutic strategies is crucial for managing DFSP.
Purpose of the Study:
- To establish and characterize a novel DFSP cell line, DFSP-DPH1, from a patient tumor.
- To investigate the molecular characteristics and drug sensitivity of the new cell line.
- To provide a valuable preclinical model for DFSP research.
Main Methods:
- DFSP cell line establishment and comprehensive characterization (morphology, marker expression, STR profiling).
- Sanger sequencing for fusion transcript identification (COL1A1::PDGFB).
- Functional assays (proliferation, spheroid formation, migration, invasion) and drug sensitivity screening (PDGFR inhibitors).
- Transcriptomic analysis to identify enriched pathways.
Main Results:
- DFSP-DPH1 cell line successfully established, retaining key features of the original tumor (spindle morphology, CD34+, vimentin+).
- Identified a novel COL1A1 exon 46-PDGFB exon 2 fusion transcript.
- DFSP-DPH1 demonstrated robust proliferation, anchorage-independent growth, migration, and invasion.
- Confirmed imatinib resistance and identified superior efficacy of other PDGFR inhibitors.
- Transcriptomic analysis revealed enriched pathways in cancer and related processes.
Conclusions:
- The novel DFSP-DPH1 cell line is a valuable preclinical model for studying imatinib-resistant DFSP.
- This model will facilitate investigations into DFSP pathogenesis, drug resistance mechanisms, and the development of new therapeutic approaches.
- DFSP-DPH1 offers a platform for evaluating novel treatment strategies against DFSP.
