ArticleClinical cancer research : an official journal of the American Association for Cancer Research2019
Identification of Rigosertib for the Treatment of Recessive Dystrophic Epidermolysis Bullosa-Associated Squamous Cell Carcinoma.
Article in Clinical cancer research : an official journal of the American Association for Cancer Research, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.
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Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it, 40 citations in OpenAlex.
- Therapies for cutaneous squamous cell carcinoma in recessive dystrophic epidermolysis bullosa: a systematic review of 157 cases.Orphanet journal of rare diseases · 2024Pooled it
- Rigosertib Reverses Hypertrophic Cardiomyopathy in Noonan Syndrome.Circulation · 2026Article
- miR-129-1-3p down-regulation promotes BAG cochaperone 3 (BAG3)-driven pro-fibrotic processes in primary fibroblasts from patients with recessive dystrophic epidermolysis bullosa.Non-coding RNA research · 2025Article
- Rigosertib as a potential therapeutic option for patients with recessive dystrophic epidermolysis bullosa squamous cell carcinoma.The British journal of dermatology · 2025Article
- CETSA-MS unveils novel targets engaged by rigosertib to promote anti-tumor activity and inflammatory responses.iScience · 2025Article
- Biomarker Discovery in Rare Malignancies: Development of a miRNA Signature for RDEB-cSCC.Cancers · 2023Article
- Lights and Shadows on the Cancer Multi-Target Inhibitor Rigosertib (ON-01910.Na).Pharmaceutics · 2023Review
- Squamous Cell Carcinoma in Patients with Inherited Epidermolysis Bullosa: Review of Current Literature.Cells · 2022Review
- Future applications of 3D bioprinting: A promising technology for treating recessive dystrophic epidermolysis bullosa.Experimental dermatology · 2022Article
- Transcriptome-Guided Drug Repurposing for Aggressive SCCs.International journal of molecular sciences · 2022Article
- Investigational Treatments for Epidermolysis Bullosa.American journal of clinical dermatology · 2021Review
- Cutaneous Squamous Cell Carcinoma in Epidermolysis Bullosa: a 28-year Retrospective Study.Acta dermato-venereologica · 2021Observational
- Impaired Wound Healing, Fibrosis, and Cancer: The Paradigm of Recessive Dystrophic Epidermolysis Bullosa.International journal of molecular sciences · 2021Review
- Translational perspectives to treat Epidermolysis bullosa-Where do we stand?Experimental dermatology · 2020Article
- Small molecule drug development for rare genodermatoses - evaluation of the current status in epidermolysis bullosa.Orphanet journal of rare diseases · 2020Review
- Epidermolysis bullosa.Nature reviews. Disease primers · 2020Review
- Molecular Therapeutics in Development for Epidermolysis Bullosa: Update 2020.Molecular diagnosis & therapy · 2020Review
- Humanization of Tumor Stroma by Tissue Engineering as a Tool to Improve Squamous Cell Carcinoma Xenograft.International journal of molecular sciences · 2020Article
- EGFR inhibition for metastasized cutaneous squamous cell carcinoma in dystrophic epidermolysis bullosa.Orphanet journal of rare diseases · 2019Article
- Epidermolysis Bullosa-Associated Squamous Cell Carcinoma: From Pathogenesis to Therapeutic Perspectives.International journal of molecular sciences · 2019Review
Corrections and comments
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Authors and funding
21 authors at 8 institutions in 7 countries.
Funding
Abstract
purposeSquamous cell carcinoma (SCC) of the skin is the leading cause of death in patients with the severe generalized form of the genetic disease recessive dystrophic epidermolysis bullosa (RDEB). Although emerging data are identifying why patients suffer this fatal complication, therapies for treatment of RDEB SCC are in urgent need.
resultsON-01910 (or rigosertib) exhibited significant specificity for RDEB SCC: in culture rigosertib induced apoptosis in 10 of 10 RDEB SCC keratinocyte populations while only slowing the growth of normal primary skin cells at doses 2 orders of magnitude higher. Furthermore, rigosertib significantly inhibited the growth of two RDEB SCC in murine xenograft studies with no apparent toxicity. Mechanistically, rigosertib has been shown to inhibit multiple signaling pathways. Comparison of PLK1 siRNA with MEK inhibition, AKT inhibition, and the microtubule-disrupting agent vinblastine in RDEB SCC shows that only PLK1 reduction exhibits a similar sensitivity profile to rigosertib.
conclusionsThese data support a "first in RDEB" phase II clinical trial of rigosertib to assess tumor targeting in patients with late stage, metastatic, and/or unresectable SCC.
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