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Molecular Research in Bone and Soft Tissue Tumors
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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 January 2026 | Viewed by 8294

Special Issue Editor

Dr. Shinji Miwa

E-Mail Website
Guest Editor
Department of Orthopedic Surgery, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan
Interests: sarcoma; chemotherapy; immunotherapy; biological reconstruction
Special Issues, Collections and Topics in MDPI journals

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 (4 papers)

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Research

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15 pages, 2374 KB  
Article
Preclinical Evaluation of Repurposed Antimalarial Artemisinins for the Treatment of Malignant Peripheral Nerve Sheath Tumors
by Heather M. Duensing, Jalen M. Dixon, Owen R. Hunter, Nicolina C. Graves, Nickalus C. Smith, Andersen J. Tomes and Cale D. Fahrenholtz
Int. J. Mol. Sci. 2025, 26(14), 6628; https://doi.org/10.3390/ijms26146628 - 10 Jul 2025
Viewed by 1536
Abstract
Malignant peripheral nerve sheath tumors (MPNSTs) are a rare type of soft tissue sarcoma associated with poor prognoses. The standard of care for non-resectable tumors consists of surgical excision followed by radiation and chemotherapy. MPNSTs are most common in patients with neurofibromatosis type [...] Read more.
Malignant peripheral nerve sheath tumors (MPNSTs) are a rare type of soft tissue sarcoma associated with poor prognoses. The standard of care for non-resectable tumors consists of surgical excision followed by radiation and chemotherapy. MPNSTs are most common in patients with neurofibromatosis type 1 but can also occur sporadically. Regardless of origin, MPNSTs most often rely on signaling pathways that increase basal oxidative stress. This provides the basis for developing therapeutics with mechanisms that can potentiate oxidative stress to selectively eradicate tumor cells at doses that are tolerable for normal cells. Artemisinin derivatives are a mainstay of malaria therapy worldwide, with a well-established safety profile. Artemisinin’s antimalarial effects are due to an endoperoxide bridge in its chemical structure that induces oxidative stress. We found that artesunate (ARS) and metabolite dihydroartemisinin (DHA) are selectively cytotoxic to MPNST cells relative to normal Schwann cells with the endoperoxide bridge required for activity. Mechanistically, DHA induced oxidative stress, lipid peroxidation, and DHA-mediated cytotoxicity could be prevented with co-administration of the antioxidant N-acetyl-cysteine. Furthermore, we found that DHA was able to selectively remove MPNST from co-culture with normal Schwann cells. These data supports the further development of artemisinins for the clinical management of MPNST. Full article
(This article belongs to the Special Issue Molecular Research in Bone and Soft Tissue Tumors)
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18 pages, 9773 KB  
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
Cited by 1 | Viewed by 1109
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 KB  
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 6 | Viewed by 4428
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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Other

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18 pages, 4718 KB  
Case Report
Single-Cell Sequencing Reveals Novel Tumor Populations and Their Interplay with the Immune Microenvironment in a Pleomorphic Rhabdomyosarcoma
by Elena E. Kopantseva, Alexander V. Ikonnikov, Maxim E. Menyailo, Timur I. Fetisov, Anastasia A. Korobeynikova, Kirill I. Kirsanov, Anastasia A. Tararykova, Beniamin Yu. Bokhyan, Nikolay A. Kozlov, Marianna G. Yakubovskaya and Evgeny V. Denisov
Int. J. Mol. Sci. 2025, 26(23), 11420; https://doi.org/10.3390/ijms262311420 - 26 Nov 2025
Viewed by 435
Abstract
Pleomorphic rhabdomyosarcoma is a rare soft-tissue tumor that occupies an uncertain middle ground between rhabdomyosarcoma and undifferentiated pleomorphic sarcoma. With its relative rarity, aggressiveness, and lack of detailed characterization, it presents a challenging task for therapeutic treatment. In this case study, we use [...] Read more.
Pleomorphic rhabdomyosarcoma is a rare soft-tissue tumor that occupies an uncertain middle ground between rhabdomyosarcoma and undifferentiated pleomorphic sarcoma. With its relative rarity, aggressiveness, and lack of detailed characterization, it presents a challenging task for therapeutic treatment. In this case study, we use single-cell transcriptomics to investigate the heterogeneous landscape of pRMS and the tumor microenvironment. We demonstrate that the tumor populations in pRMS have a clear division into myogenic and non-myogenic clusters, with the non-myogenic clusters having more numerous communication links with the immune populations. All pRMS tumor clusters use the MIF-CD74 pathway to suppress the immune response, while APP, PTN, and CXCL12 signaling are employed predominantly by the non-myogenic tumor clusters. The cytotoxic T cells in pRMS bear markers of exhaustion (LAG3, HAVCR2, EOMES), and the macrophages express myeloid checkpoint-related genes (SIGLEC1, SIRPA, CSF1R, HAVCR2). This transcriptomic data suggests that targeting MIF and APP signaling in pRMS may have therapeutic potential; however, studies on multiple-patient cohorts, protein verification, and in vitro and in vivo validation are still needed for clinical actionability. Full article
(This article belongs to the Special Issue Molecular Research in Bone and Soft Tissue Tumors)
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