Abstract
Rhabdomyosarcoma (RMS) is the most frequent pediatric soft-tissue tumor accounting for about 7% of childhood malignancies. Multimodal therapy is the standard treatment for individuals with RMS but generally fails to cure high-risk group patients and can result in long-term side effects. Therefore, understanding the mechanisms driving RMS might help to find new candidate targets for more specific and effective therapeutic modalities. One of the molecular machineries which is often deregulated in cancer and specifically involved in tumorigenesis of RMS, is Hedgehog (Hh) signaling. There is increasing evidence that targeting this developmental pathway may hold promise in future treatment strategies for RMS. In this review, we discuss the contribution of the Hh pathway in RMS, the challenges of inhibiting this embryonic signaling in children with an update on recent preclinical data and ongoing clinical trials.
Keywords: Pediatric cancers, rhabdomyosarcoma, developmental pathways, hedgehog signaling, mouse models, targeted therapy.
Current Drug Targets
Title:Interfering with Hedgehog Pathway: New Avenues for Targeted Therapy in Rhabdomyosarcoma
Volume: 17 Issue: 11
Author(s): Gabriele Manzella and Beat W. Schäfer
Affiliation:
Keywords: Pediatric cancers, rhabdomyosarcoma, developmental pathways, hedgehog signaling, mouse models, targeted therapy.
Abstract: Rhabdomyosarcoma (RMS) is the most frequent pediatric soft-tissue tumor accounting for about 7% of childhood malignancies. Multimodal therapy is the standard treatment for individuals with RMS but generally fails to cure high-risk group patients and can result in long-term side effects. Therefore, understanding the mechanisms driving RMS might help to find new candidate targets for more specific and effective therapeutic modalities. One of the molecular machineries which is often deregulated in cancer and specifically involved in tumorigenesis of RMS, is Hedgehog (Hh) signaling. There is increasing evidence that targeting this developmental pathway may hold promise in future treatment strategies for RMS. In this review, we discuss the contribution of the Hh pathway in RMS, the challenges of inhibiting this embryonic signaling in children with an update on recent preclinical data and ongoing clinical trials.
Export Options
About this article
Cite this article as:
Manzella Gabriele and Schäfer W. Beat, Interfering with Hedgehog Pathway: New Avenues for Targeted Therapy in Rhabdomyosarcoma, Current Drug Targets 2016; 17 (11) . https://dx.doi.org/10.2174/1389450116666150505122604
DOI https://dx.doi.org/10.2174/1389450116666150505122604 |
Print ISSN 1389-4501 |
Publisher Name Bentham Science Publisher |
Online ISSN 1873-5592 |
Call for Papers in Thematic Issues
New drug therapy for eye diseases
Eyesight is one of the most critical senses, accounting for over 80% of our perceptions. Our quality of life might be significantly affected by eye disease, including glaucoma, diabetic retinopathy, dry eye, etc. Although the development of microinvasive ocular surgery reduces surgical complications and improves overall outcomes, medication therapy is ...read more
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
Related Articles
-
Management of Glioblastoma Multiforme by Phytochemicals: Applications of Nanoparticle-Based Targeted Drug Delivery System
Current Drug Targets mTOR Inhibition and the Tumor Vasculature
Current Angiogenesis (Discontinued) FOXO Transcription Factors and their Role in Disorders of the Female Reproductive Tract
Current Drug Targets Irreversible LSD1 Inhibitors: Application of Tranylcypromine and Its Derivatives in Cancer Treatment
Current Topics in Medicinal Chemistry Proteomic Approaches for the Study of Transgelins as Tumor-associated Proteins and Potential Biomarkers
Current Proteomics The Critical Role of Vascular Endothelial Growth Factor in Tumor Angiogenesis
Current Cancer Drug Targets Peptides to Target Tumor Vasculature and Lymphatics for Improved Anti-Angiogenesis Therapy
Current Cancer Drug Targets CXCL12-CXCR4 Axis in Angiogenesis, Metastasis and Stem Cell Mobilization
Current Pharmaceutical Design Cyclodepsipeptides - Potential Drugs and Lead Compounds in the Drug Development Process
Current Medicinal Chemistry CXCR4-CXCL12-Dependent Inflammatory Network and Endothelial Progenitors
Current Medicinal Chemistry A Dilemma of Functional Genomics: Count the Chickens or Study their Eggs ?
Current Genomics CD44 and EpCAM: Cancer-Initiating Cell Markers
Current Molecular Medicine Retinoids as Differentiating Agents in Oncology: A Network of Interactions with Intracellular Pathways as the Basis for Rational Therapeutic Combinations
Current Pharmaceutical Design Natural Products as Anticancer Agents
Current Drug Targets Urotensin-II Receptor Antagonists
Current Medicinal Chemistry Gynecomastia in Infants, Children, and Adolescents
Recent Patents on Endocrine, Metabolic & Immune Drug Discovery Magnetic Resonance Imaging of Nasopharyngeal Adenoid Hypertrophy in Children Following Bone Marrow Transplantation
Current Medical Imaging A Progressive Review of V600E-B-RAF-Dependent Melanoma and Drugs Inhibiting It
Mini-Reviews in Medicinal Chemistry Targeting EZH2 for Cancer Therapy: Progress and Perspective
Current Protein & Peptide Science Tumor-related Molecular Regulatory Mechanisms of Long Non-coding RNA RMST: Recent Evidence
Mini-Reviews in Medicinal Chemistry