Molecular pathways and therapeutic strategies in dermatofibrosarcoma protuberans (DFSP)

unravelling the tumor’s genetic landscape

Authors

  • Harpreet Singh School of Pharmaceutical Sciences, IFTM University, Moradabad, Uttar Pradesh, 244102, India. E-mail: harpreetproctor@rediffmail.com https://orcid.org/0000-0001-5229-6569
  • Heena Bholaram Choudhary Department of Pharmacology, BVDU, Poona College of Pharmacy, Pune, 411038, Maharashtra, India
  • Deepa Satish Mandlik Department of Pharmacology, BVDU, Poona College of Pharmacy, Pune, 411038, Maharashtra, India https://orcid.org/0000-0001-8505-7038
  • Manoj Subhash Magre Department of Pharmacology, BVDU, Poona College of Pharmacy, Pune, 411038, Maharashtra, India https://orcid.org/0009-0000-3503-3222
  • Sourav Mohanto Department of Pharmaceutics, Yenepoya Pharmacy College & Research Centre, Yenepoya (Deemed to be University), Mangalore, Karnataka, 575018, India https://orcid.org/0000-0001-5599-5445
  • Mohammed Gulzar Ahmed Department of Pharmaceutics, Yenepoya Pharmacy College & Research Centre, Yenepoya (Deemed to be University), Mangalore, Karnataka, 575018, India https://orcid.org/0000-0002-9226-9984
  • Bhuvnesh Kumar Singh Faculty of Pharmacy, Moradabad Educational Trust, Moradabad, Uttar Pradesh, 244001, India https://orcid.org/0000-0001-7264-337X
  • Arun Kumar Mishra SOS School of Pharmacy, IFTM University, Moradabad, Uttar Pradesh, 244102, India https://orcid.org/0000-0002-8569-988X
  • Arvind Kumar School of Pharmaceutical Sciences, IFTM University, Moradabad, Uttar Pradesh, 244102, India https://orcid.org/0000-0003-4754-5318
  • Amrita Mishra School of Pharmaceutical Sciences, Delhi Pharmaceutical Sciences and Research University, New Delhi, 110017, India
  • T. Venkatachalam Department of Pharmaceutical Chemistry, JKKMMRFs-Annai JKK Sampoorani Ammal College of Pharmacy, Komarapalayam, The Tamil Nadu Dr. MGR Medical University, Chennai, Tamil Nadu, 638183, India https://orcid.org/0000-0002-9897-5775
  • Hitesh Chopra Department of Biosciences, Saveetha School of Engineering, Saveetha Institute of Medical and Technical Sciences, Chennai - 602105, Tamil Nadu, India https://orcid.org/0000-0001-8867-7603

DOI:

https://doi.org/10.17179/excli2024-7164

Keywords:

Dermatofibrosarcoma Protuberans, DFSP, cancer, genome, immunotherapy

Abstract

Dermatofibrosarcoma Protuberans (DFSP) is a rare soft tissue sarcoma distinguished by its infiltrative growth pattern and recurrence potential. Understanding the molecular characteristics of DFSP is essential for enhancing its diagnosis, prognosis, and treatment strategies. The paper provides an overview of DFSP, highlighting the significance of its molecular understanding. The gene expression profiling has uncovered unique molecular signatures in DFSP, highlighting its heterogeneity and potential therapeutic targets. The Platelet-Derived Growth Factor Receptors (PDGFRs) and Fibroblast Growth Factor Receptors (FGFRs) signaling pathways play essential roles in the progression and development of DFSP. The abnormal activation of these pathways presents opportunities for therapeutic interventions. Several emerging therapies, i.e., immunotherapies, immunomodulatory strategies, and immune checkpoint inhibitors, offer promising alternatives to surgical resection. In DFSP management, combination strategies, including rational combination therapies, aim to exploit the synergistic effects and overcome resistance. The article consisting future perspectives and challenges includes the discovery of prognostic and predictive biomarkers to improve risk stratification and treatment selection. Preclinical models, such as Patient-derived xenografts (PDX) and genetically engineered mouse models, help study the biology of DFSP and evaluate therapeutic interventions. The manuscript also covers small-molecule inhibitors, clinical trials, immune checkpoint inhibitors for DFSP treatment, combination therapies, rational therapies, and resistance mechanisms, which are unique and not broadly covered in recent pieces of literature.

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Published

2024-05-14

How to Cite

Singh, H., Choudhary, H. B., Mandlik, D. S., Magre, M. S., Mohanto, S., Ahmed, M. G., … Chopra, H. (2024). Molecular pathways and therapeutic strategies in dermatofibrosarcoma protuberans (DFSP): unravelling the tumor’s genetic landscape. EXCLI Journal, 23, 727–762. https://doi.org/10.17179/excli2024-7164

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