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Molecular Research in Bone and Soft Tissue Tumors

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Oncology".

Deadline for manuscript submissions: 20 June 2025 | Viewed by 1730

Special Issue Editor

Special Issue Information

Dear Colleagues,

Although the developments in multidisciplinary treatment have significantly improved clinical outcomes in bone and soft-tissue sarcomas, the outcomes of patients with metastatic/recurrent sarcoma remain poor. Therefore, novel systemic treatments including molecular targeting agents and immunotherapy are needed in order to improve outcomes. Recently, there has been a large number of studies on therapeutic targets and clinical trials for molecular targeting agents in bone and soft-tissue sarcomas. Furthermore, immune checkpoint inhibitors have shown superior outcomes compared to standard chemotherapy in several types of malignancies. This Special Issue aims to bring together high-quality original/review articles on basic and clinical studies into bone and soft-tissue sarcomas. Molecular biology, microenvironments, and mechanisms of metastasis, as well as articles on clinical research into new anticancer agents and immunotherapy, are welcome.

Dr. Shinji Miwa
Guest Editor

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Keywords

  • sarcoma
  • therapeutic target
  • metastasis
  • anticancer agent
  • immunotherapy

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Published Papers (2 papers)

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Research

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18 pages, 9773 KiB  
Article
Cucurbitacin B Exhibits Antitumor Effects on Chordoma Cells via Disruption of Brachyury
by Carolin Seeling, Johannes Neumahr, Fabian Häberle, André Lechel, Peter Möller, Nadine T. Gaisa, Thomas F. E. Barth and Kevin Mellert
Int. J. Mol. Sci. 2025, 26(8), 3864; https://doi.org/10.3390/ijms26083864 - 18 Apr 2025
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Abstract
Chordomas are rare malignant tumors of the bone, originating from remnants of notochordal cells. The transcription factor brachyury, encoded by TBXT, serves as a critical diagnostic marker and is essential for tumor growth. While brachyury’s role in regulating the cytoskeleton during embryogenesis [...] Read more.
Chordomas are rare malignant tumors of the bone, originating from remnants of notochordal cells. The transcription factor brachyury, encoded by TBXT, serves as a critical diagnostic marker and is essential for tumor growth. While brachyury’s role in regulating the cytoskeleton during embryogenesis and tumorigenesis is well understood, the reverse—whether cytoskeletal alterations can influence brachyury levels—remains unclear. Despite advances in understanding chordoma biology, there are currently no approved targeted therapies, underscoring the need for novel therapeutic approaches. Three chordoma cell lines were treated with cytoskeletal inhibitors, including the actin-targeting compounds Cucurbitacin B (CuB) and Latrunculin B (LatB). Morphological changes, TBXT expression, and cell viability were analyzed. The effects of CuB were examined over time and across concentrations, with cell viability assessed via apoptosis and cytotoxicity assays. Microarray gene expression profiling of ten chordoma cell lines was performed to explore CuB-mediated transcriptional changes. Rescue experiments using a TBXT open reading frame vector and co-treatments with autophagy and proteasome inhibitors were conducted to elucidate the mechanisms of brachyury depletion. Both CuB and LatB induced significant morphological changes, but only CuB caused near-complete depletion of brachyury. This effect was time- and concentration-dependent, correlating with reduced cell viability driven primarily by apoptosis. Microarray analysis revealed that CuB treatment upregulated protein refolding pathways and downregulated protein glycosylation. Notably, TBXT transcription was only slightly suppressed, indicating that brachyury depletion was largely post-transcriptional. Rescue experiments and co-treatments implicated dysregulated protein refolding and endoplasmic reticulum (ER) stress as key mechanisms underlying CuB-mediated brachyury loss. This study demonstrates that actin cytoskeleton disruption by CuB depletes brachyury in chordoma cells, primarily through dysregulated protein refolding and ER stress rather than transcriptional repression. These findings suggest that targeting actin cytoskeleton dynamics or protein unfolding pathways may provide novel therapeutic approaches for chordoma treatment. Full article
(This article belongs to the Special Issue Molecular Research in Bone and Soft Tissue Tumors)
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Review

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25 pages, 1216 KiB  
Review
Chondrosarcoma: New Molecular Insights, Challenges in Near-Patient Preclinical Modeling, and Therapeutic Approaches
by Lorena Landuzzi, Francesca Ruzzi, Pier-Luigi Lollini and Katia Scotlandi
Int. J. Mol. Sci. 2025, 26(4), 1542; https://doi.org/10.3390/ijms26041542 - 12 Feb 2025
Cited by 1 | Viewed by 991
Abstract
Chondrosarcoma (CS), the second most common malignant bone tumor after osteosarcoma, accounts for 20–30% of all malignant bone tumors. It mainly affects adults, middle-aged, and elderly people. The CS family includes various entities displaying peculiar biological, genetic, and epigenetic characteristics and clinical behaviors. [...] Read more.
Chondrosarcoma (CS), the second most common malignant bone tumor after osteosarcoma, accounts for 20–30% of all malignant bone tumors. It mainly affects adults, middle-aged, and elderly people. The CS family includes various entities displaying peculiar biological, genetic, and epigenetic characteristics and clinical behaviors. Conventional CS is the most common subtype. High-grade, dedifferentiated, and mesenchymal CS, as well as unresectable and metastatic CS, exhibit poor prognoses due to their intrinsic resistance to radiotherapy and chemotherapy, underscoring the urgent need for novel therapeutic strategies. CS research is dealing with several challenges. Experimental studies can rely on animal and patient-derived models, but the paucity of representative near-patient preclinical models has hampered predictive drug screening research. This review describes the main clinical and molecular features of CS subtypes, discussing recent data on the genetic alterations and molecular mechanisms involved in CS pathogenesis and progression. The review provides an overview of the current in vitro and in vivo CS models, discusses their advantages and limitations, and highlights the recent efforts in the development of new targeted therapies against CS dependencies, including IDH1/2 mutations, NAD+ dependency, and SIRT1-HIF-2α axis, or exploring DR5 targeting, antiangiogenic therapies, epigenetic drugs, and immunological approaches. All such strategies, in combination with advanced preclinical modeling and personalized multi-omic profiling, hold promise for improving the survival of patients with advanced CS. Full article
(This article belongs to the Special Issue Molecular Research in Bone and Soft Tissue Tumors)
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