RARE DISEASE
Chondrosarcoma
Chondrosarcoma
Chondrosarcoma
Drug discovery
6
drugs
With orphan designations
Overview
Chondrosarcoma is a malignant bone tumor arising from cartilage-producing cells, representing ~10% of primary bone cancers [6][9]. It predominantly affects adults aged 40-75 (mean 51) [9][12], with 1-3 cases per 200,000 annually [5][7][9]. Conventional subtypes (85% of cases) grow slowly, while rare variants like dedifferentiated (10%) and mesenchymal chondrosarcoma show aggressive behavior [5][9]. Diagnosis requires histopathology and imaging correlation [6], with surgical resection remaining the cornerstone of treatment due to inherent chemoresistance and radioresistance [3][5][8].
Categories: rare bone diseases, rare neoplastic diseases
Research Papers
2,299 drug discovery papers about Chondrosarcoma, with 5 first-in-class and 4 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
2,299 drug discovery papers about Chondrosarcoma, with 5 first-in-class and 4 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
categories:
Small molecules
cell therapies
2026-09-25 | [Resection of a chondrosarcoma of the first metacarpal and reconstruction using composite osteosynthesis : Case report and literature review].
Chondrosarcoma is the most common malignant bone tumor of the hand. As conventional chondrosarcomas generally respond poorly to radiotherapy and chemotherapy, surgical resection is crucial. A 76-year-old woman with a chondrosarcoma of the first metacarpal underwent 2‑stage reconstruction using composite osteosynthesis after tumor resection. After 18 months the functional outcome was satisfactory, with no local recurrence, metastases or implant-associated complications. Composite osteosynthesis can represent a function-preserving reconstructive option in selected patients.
2026-09-23 | Restoring thoracic stability with rigid titanium rib reconstruction after radical forequarter and upper chest wall resection for T4 chondrosarcoma.
Extensive anterior chest wall involvement in chondrosarcoma presents major reconstructive challenges following radical oncologic resection. A 55-year-old man with cT4N0M0 chondrosarcoma involving the right shoulder girdle, ribs 1-4, and right upper lobe underwent en bloc extended forequarter amputation, multilevel rib resection, and right upper lobe segmentectomy. Chest wall reconstruction was performed using a titanium-based RibLoc plating system with vascularized soft-tissue flap coverage. Postoperatively, chest wall stability was preserved without paradoxical motion, and radiographs confirmed appropriate implant positioning. The patient was successfully weaned from mechanical ventilation on postoperative day two and transferred from the intensive care unit on day three without major complications. Rigid titanium rib fixation provided effective structural restoration and favorable early postoperative outcomes following extensive anterior chest wall resection for advanced chondrosarcoma.
2026-07-10 | Short-term Functional Outcomes of Shoulder Girdle Limb Salvage in Primary Bone Tumors: A 2-year Follow-up Study.
The shoulder girdle, encompassing the proximal humerus, scapula, lateral third of the clavicle, and surrounding soft tissues, is the third most frequent site for bone tumors. The proximal humerus is the most commonly affected area, followed by the scapula and clavicle. Managing malignant or aggressive benign bone tumors in this region is particularly challenging due to the proximity to vital neurovascular structures and the need to maintain both stability and dexterity in the shoulder. While traditional treatment often involved extensive resections, contemporary limb salvage techniques focus on preserving function while ensuring effective tumor control. This prospective study included 18 patients who underwent various limb salvage procedures at the Department of Orthopaedics, Vydehi Institute of Medical Sciences and Research Centre in Bengaluru between 2012 and 2019, with a minimum follow-up of 2 years. Diagnosis and staging were performed using imaging modalities and core needle biopsies, with musculoskeletal tumors staged according to the Enneking System. Functional outcomes were assessed using the Musculoskeletal Tumor Society Rating Scale (MSTS). Surgical techniques varied and included endoprosthetic reconstruction, arthrodesis with fibular grafting and plating, and plate fixation with primary shortening. Among the 18 patients, the majority (61%) had giant cell tumors, followed by aneurysmal bone cysts (17%), chondrosarcoma (11%), and osteosarcoma (11%). Functional outcomes, as measured by the MSTS, showed a 75% satisfactory rate. The study population was composed of 55.56% males and 44.44% females. Key parameters evaluated included pain, functional activity, hand positioning, dexterity, lifting ability, and emotional acceptance. Limb salvage surgery for primary bone tumors of the shoulder girdle offers favorable functional outcomes and represents a viable alternative to amputation. Modern reconstruction techniques, such as endoprosthetic reconstruction and fibular grafting with plating, achieve satisfactory results in both function and appearance. The MSTS scoring system is an effective tool for assessing postoperative functional status, and there is a growing preference for less aggressive surgical techniques that preserve limb function while ensuring tumor control.
2026-07-02 | Identification of immunopeptides (pHLA) as candidate therapeutic targets in chondrosarcoma
Background Chondrosarcoma (CHS) is the second most common primary malignant bone tumor and remains resistant to conventional therapies, underscoring the need to discover novel therapeutic targets. Cancer/testis antigens (CTAs), a class of tumor-associated proteins, represent attractive antigens for cancer immunotherapies such as adoptive T cell therapy. However, the expression profile of CTAs and their associated targetable immunopeptides presented in the Human Leukocyte Antigen-I context (pHLA) remain unknown in CHS. This study aims to characterize the CTA expression profile according to the tumor immune phenotype and clinical outcomes, and to identify the most relevant pHLA to target in CHS. Method We analyzed the CTA expression profile in tumors from 63 conventional CHS patients and in healthy tissues using GTEx and HPA databases to identify CHS-associated CTAs. Cox proportional hazards models combined with hierarchical clustering were used to correlate CTA expression with the overall survival of patients. The tumor immune phenotype was estimated based on immune gene expression signatures using a deconvolution method and a Pearson correlation coefficient matrix. The CTA-derived pHLA were characterized using immunopeptidomic profiling based on HLA-I immunoprecipitation and mass spectrometry in grade 2 and 3 CHS models. NetMHC was used to predict the binding affinity of identified pHLA to the HLA-A*02:01 and HLA-A*01:01 alleles. Results We identified a poor prognosis CTA signature predominantly associated with a non-inflamed tumor immunophenotype. Immunopeptidomic profiling revealed broad pHLA repertoires, including previously well-characterized CTAs from PRAME, CTAG2 and the MAGE-A family, as well as newly identified candidates with strong predicted HLA-binding affinity from CTAs such as HHIPL2, DBF4, BRIP1, CBX2 and DIAPH3. Conclusion This study provides the first atlas of pHLA in CHS and suggests specific antigenic targets for TCR-T cell therapies and targeted therapies such as antibody-drug conjugates.
2026-06-12 | Robot-Assisted "Postage-Stamp" Vertebrotomy for Spinal Tumor Resection: Case Report.
Background: Achieving adequate oncological margins in tumors involving the thoracic costovertebral junction is technically challenging because of complex regional anatomy and the need to preserve neurological and biomechanical integrity. This case report describes a robot-assisted margin-extension strategy after incomplete resection of a thoracic costovertebral chondrosarcoma. Methods: A 31-year-old man with grade 1 chondrosarcoma of the left sixth rib underwent second-stage surgical radicalization after prior incomplete resection with positive medial margins. Following multidisciplinary discussion, a single-stage posterior procedure was performed, including robot-assisted T4-T8 stabilization with radiolucent CFR-PEEK instrumentation and robot-assisted sagittal vertebral osteotomy ("Postage-Stamp Osteotomy") of T6 to achieve en bloc removal of the involved costovertebral segment. Results: The osteotomy was executed using a pedicle-referenced robotic trajectory workflow with sequential navigated drilling and controlled completion with a navigated osteotome. Total operative time was 379 min, with estimated blood loss of 800 mL. No major intraoperative neurovascular complications occurred. Histopathology confirmed negative margins. The patient was mobilized on postoperative day 1 and discharged on postoperative day 6 without new neurological deficits. Radiological follow-up at 3 months showed no recurrence, while clinical follow-up at 5 months demonstrated full return to baseline activities. Conclusions: This report describes a technically feasible robot-assisted margin-extension strategy in a highly selected thoracic spinal oncology scenario. Although long-term oncological conclusions cannot be drawn from a single case, tailored technology-enabled margin-oriented approaches may represent a case-specific option in carefully selected patients.
antibodies
2026-09-24 | Radiological and multidisciplinary management of extraskeletal myxoid chondrosarcoma: A case report.
Extraskeletal myxoid chondrosarcoma (EMC), a very rare subtype of soft tissue sarcoma (STS), distinguished by its histopathological profile, has unique clinical prognosis of potential for late local recurrences and distant metastases due to its molecular alterations. The infrequent occurrence of EMC, coupled with its radiological characteristics that often overlap with those of other myxoid-rich neoplasms, may impose considerable diagnostic difficulties for clinicians. A 43-year-old male who presented with a progressively enlarging mass in the distal right thigh and knee region since six-month prior. Initial imaging studies, including computed tomography (CT) and non-contrast magnetic resonance imaging (MRI), suggested a diagnosis of liposarcoma. However, further evaluation including a comprehensive contrast-enhanced MRI revealed a large multilobulated, well-defined soft tissue mass at the distal anteromedial femur. The lesion demonstrated isointense to hypointense signal on T1-weighted sequence and hyperintensity on T2-weighted/STIR sequences reflecting myxoid matrix, and prominent peripheral and septal enhancement post-gadolinium administration. The mass also exhibited local bone erosion and infiltration into surrounding musculature and the knee joint. A core needle biopsy followed by immunohistochemical profiling was consistent with a diagnosis of EMC. Following multidisciplinary consultation, the patient underwent successful limb salvage surgery, involving wide excision of the tumor and reconstruction using prosthesis. This case underscores the critical role of advanced imaging techniques in characterizing soft tissue masses and suggesting EMC in the differential diagnosis prior to histopathological confirmation. Moreover, multidisciplinary approach for the effective management of this rare sarcoma, including complex surgical reconstruction to preserve limb function is essential.
2026-09-18 | Emerging therapeutic strategies and clinical development of investigational agents for conventional chondrosarcoma.
Conventional chondrosarcoma (cCS) is a malignant bone tumor with intrinsic resistance to cytotoxic therapy. Advances in genomic profiling have revealed recurrent alterations that are redefining the therapeutic landscape for these tumors. Literature searches of PubMed and ClinicalTrials.gov for the terms conventional chondrosarcoma, systemic therapy, targeted therapy, immunotherapy, chemotherapy, molecular targets, and clinical trial through June 2026 were performed. Clinical and translational developments regarding treatment of cCS were summarized, emphasizing the following emerging targeted and immunotherapeutic agents: isocitrate dehydrogenase 1 and 2 (IDH1/2) inhibitors, DR5 agonists, methylthioadenosine phosphorylase (MTAP)-deficient PRMT5 inhibitors, TP53 reactivators, histone deacetylase and BCL-2 modulators, immunotherapy approaches, multi-targeted tyrosine kinase inhibitors (TKIs) and antibody-drug conjugates. Multi-targeted TKIs remain the most commonly used systemic therapies for advanced cCS; however, recent advances in DR5 agonism and IDH1 inhibition provide proof of concept that biologically informed therapeutic strategies can achieve meaningful clinical benefit. Continued progress will require comprehensive molecular profiling, rational combination strategies, and innovative clinical trial designs to accelerate drug development in this rare malignancy.
2026-08-31 | Primary Mesenchymal Chondrosarcoma of the Spine Masquerading as Potts Spine: A Case Report
Background: Primary mesenchymal chondrosarcoma is a rare malignant neoplasm originating from bone and soft tissue commonly involving maxilla and mandible, but can also involve ribs, pelvis, femur, and spine. Case report: 16 years-old-female presented with weakness and paresthesia of lower limbs since 15 days with difficulty in walking. CT scan showed pre and paravertebral soft tissue density lesion extending from D8 to D12 vertebral body levels. MRI showed heterogenous lesion with hypo and hyperintense areas in T1W1 and hyperintense on T2. Tumor is excised and on histopathological examination revealed the features of mesenchymal chondrosarcoma. Conclusion: Mesenchymal chondrosarcoma is a rare malignant mesenchymal tumor located in bone, intracranial sites or soft tissue with non-specific findings on imaging. Histopathology and additional immunohistochemistry is gold standard for diagnosis.
2026-06-25 | Chondrosarcoma of the Mandible: A Clinicopathological Case Report based Insight
Chondrosarcoma is a malignant mesenchymal tumour characterized by the production of cartilaginous matrix. Its occurrence within the maxillofacial region is uncommon and frequently presents a diagnostic dilemma because its clinical and radiographic features can resemble other primary bone malignancies. A 70-year-old male presented with a gradually enlarging and painful swelling involving the left side of the mandible over a period of approximately six months. Clinical examination revealed a diffuse extraoral swelling along with an intraoral exophytic and ulcerated lesion in the posterior alveolar region. Imaging studies demonstrated irregular radiopaque areas associated with cortical destruction and a characteristic sunburst-type periosteal reaction. Histopathological examination showed atypical chondrocytes embedded within a chondroid matrix, along with areas of calcification and cellular pleomorphism, confirming the diagnosis of chondrosarcoma. This case highlights the aggressive nature of mandibular chondrosarcoma and emphasizes the importance of correlating clinical, radiographic, and histopathological findings to arrive at a definitive diagnosis and guide appropriate treatment.
2026-05-27 | Pembrolizumab for MSI-high/TMB-high bone and soft tissue sarcomas: a retrospective single-institution study.
Bone and soft tissue sarcomas are rare, heterogeneous malignancies with limited treatment options for advanced stages. Pembrolizumab, an anti-PD-1 immune checkpoint inhibitor (ICI), is approved for tumors exhibiting high microsatellite instability (MSI-high) or high tumor mutation burden (TMB-high); however, its efficacy remains unclear. We reviewed records of 40 patients with advanced/recurrent sarcoma who underwent genomic profiling. MSI status and TMB were assessed using the NCC Oncopanel® or FoundationOne®. Patients identified as MSI-high and/or TMB-high received pembrolizumab (200 mg/body weight every 3 weeks). Clinical outcomes were evaluated using RECIST v1.1 and adverse events graded per CTCAE v5.0. Three patients (7.5%) were MSI-high and/or TMB-high: one each with undifferentiated pleomorphic bone sarcoma, leiomyosarcoma, and dedifferentiated chondrosarcoma. All had metastatic disease and received chemotherapy. Pembrolizumab was administered for 5-25 cycles for 5-18 months. Two patients achieved a partial response, and one had stable disease, yielding an objective response rate of 67% and a mean response duration of 12.7 months. No complete response or disease progression occurred. The adverse events were limited to grade 2 thyroid dysfunction and skin toxicity. No grade ≥3 events or treatment discontinuations occurred. Pembrolizumab demonstrated promising efficacy and tolerability in MSI-high/TMB-high sarcomas. These findings support biomarker-driven immunotherapy in patients with sarcomas. Comprehensive genomic profiling should be considered in refractory cases. Further studies are warranted to validate these results and explore additional biomarkers.
proteins
2026-09-07 | A logic-gated synthetic circuit coupled with microenvironmental reprogramming overcomes immune exclusion in antigen-poor chondrosarcoma.
Chondrosarcoma remains refractory to immunotherapy due to a paucity of targetable antigens and a suppressive tumor microenvironment (TME). We developed a modular strategy integrating tumor-intrinsic genetic logic with rational TME reprogramming to overcome these barriers. Using a 57,715-element massively parallel reporter assay, we identified chondrosarcoma-selective synthetic promoters and engineered a Boolean AND-gate circuit that ensures high-fidelity expression of a surface T cell engager exclusively within malignant cells. To further address the immunosuppressive TME, we identified macrophage migration inhibitory factor (MIF) as a target for blockade. Single-cell RNA sequencing in human PBMC-reconstituted chondrosarcoma xenograft models revealed that the circuit initiates T cell activation, while MIF blockade prevents exhaustion by promoting proliferative T cells and M1-polarized macrophages. Local administration induced a systemic abscopal effect, regressing distant, untreated tumors. Collectively, these findings provide a programmable blueprint for converting immunologically cold, antigen-poor malignancies into systemically immune-responsive states.
2026-05-21 | Analysis code and notebooks for: Identification of immunopeptides (pHLA) as candidate therapeutic targets in chondrosarcoma
This repository contains the analysis code and notebooks associated with the study on cancer/testis antigens (CTAs) and immunopeptides (pHLA) in chondrosarcoma (CHS). It includes transcriptomic analyses of CTA expression profiles using public databases (GTEx, HPA, E-MTAB-7264), survival analyses (Cox models and hierarchical clustering), tumor immune phenotype deconvolution, and immunopeptidomic data processing. Key findings include the identification of a poor prognosis CTA signature associated with low immune cell infiltration, and the characterization of pHLA repertoires in CHS cell lines, with the identification of novel candidate therapeutic targets.
2026-03-14 | Vasculogenic Mimicry: A Potential Therapeutic Target for Chondrosarcoma Therapy.
Chondrosarcomas (ChSs) are mesenchymal chemo- and radiation-resistant tumors, representing the second most frequently diagnosed bone sarcoma after osteosarcoma and 20% of all bone sarcomas. Most of ChS patients have a good prognosis after complete surgical resection. Conversely, patients with inoperable disease, due to the tumor location or metastatic dissemination, represent a great clinical challenge due to the lack of effective therapeutic options. In this study, to the best of our knowledge, we document, for the first time in human ChS tissues, the existence of CD-31- and Podoplanin-negative vascular-like channels containing red blood cells, allowing us to hypothesize the occurrence of vasculogenic mimicry (VM) in ChSs. By using patient-derived ChS cells and a stabilized ChS cell line, we demonstrate that ChS cells are able to form in vitro tubules apparently similar to those formed by endothelial cells. Further characterization of these vessels revealed the pivotal role of the Urokinase Plasminogen Activator Receptor (uPAR) in mediating the capability of ChS cells to form VM. Finally, we provide evidence that, unlike bevacizumab, which did not exert any effect, the uPAR-derived antiangiogenic peptide RI-3 behaves as a potent inhibitor of VM.
2025-07-05 | Pediatric mesenchymal chondrosarcoma with archetypal histology in the absence of HEY1::NCOA2 gene fusion: a molecular-morphological paradox.
Mesenchymal chondrosarcoma (MC) is a rare, aggressive malignancy characterized by a distinctive biphasic histological pattern and frequently associated with the HEY1::NCOA2 fusion gene. We report a case of a 17-year-old male presenting with a mandibular mass initially suspected to be a benign chondro-osseous lesion. Following segmental mandibulectomy, histopathological examination revealed classical features of MC, including undifferentiated small round to spindle cells arranged around staghorn vasculature and islands of mature hyaline cartilage. Immunohistochemistry showed strong S100 positivity in the cartilaginous component and diffuse NKX3.1 expression in the undifferentiated areas. However, molecular analysis via next-generation sequencing did not detect HEY1::NCOA2 fusion, raising the possibility of a fusion-negative variant. This case highlights the potential for molecular heterogeneity in MC and emphasizes that, in the presence of unequivocal morphology, absence of canonical gene fusions should not preclude the diagnosis. It reinforces the enduring role of histopathology and the need for expanded molecular profiling in such diagnostically challenging cases.
2025-04-15 | Protein Phosphatase 1 Regulatory Subunit 3C integrates cholesterol metabolism and isocitrate dehydrogenase in chondrocytes and neoplasia.
Enchondromas are common bone tumors composed of chondrocytes originating from growth plate cells which can progress to malignant chondrosarcoma. Mutations in the genes encoding isocitrate dehydrogenase (IDH1 and IDH2) are identified in a large proportion of these tumors. IDH enzymes convert isocitrate to alpha-ketoglutarate (α-KG), an essential component of the citric acid cycle. While mutant IDH enzymes produce 2-hydroxyglutarate, which has epigenetic effects important in tumor initiation, cell maintenance and growth rely on additional factors. Prior work shows that intracellular cholesterol and glycogen are upregulated in mutant IDH chondrocytes. Here, we show that Protein Phosphatase 1 Regulatory Subunit 3C (PPP1R3C, previously termed Protein Targeting to Glycogen or PTG) is highly expressed in chondrocytes harboring a mutant IDH. Furthermore, Sterol Regulatory Element-Binding Proteins (SREBPs), transcriptional regulators of sterol biosynthesis, regulate PPP1R3C expression. We found that PPP1R3C regulates glycolysis and glycolytic capacity in chondrocytes. Depletion of PPP1R3C in mouse chondrocytes in vivo suppresses the neoplastic phenotype. The growth plate phenotype associated with the genetic inhibition of cholesterol biosynthesis is partially rescued by PPP1R3C overexpression. Taken together, our data show that PPP1R3C integrates cholesterol metabolism and isocitrate dehydrogenase in growth plate and neoplastic chondrocyte metabolism by regulating intracellular glycogen levels.
small molecules
2026-09-11 | Targeting IDH1: Long-Term Efficacy and Tolerance of Ivosidenib in Advanced Chondrosarcoma.
Around 20% of primary bone malignancies are chondrosarcomas. Advanced or recurrent conventional chondrosarcomas are typically resistant to chemotherapy and radiotherapy and overall survival remains poor, highlighting the need for other therapeutic options. We present two cases of metastatic chondrosarcoma with isocitrate dehydrogenase 1 (IDH1) mutation treated with ivosidenib. In case 1, a 70-year-old woman with recurrent metastatic grade 2 chondrosarcoma achieved well-tolerated, durable stable disease for 12 months in second line (no response to conventional first line chemotherapy). In case 2, a 63-year-old man with recurrence of grade 2 chondrosarcoma maintained stable disease for 16 months on ivosidenib first line. In both cases, there were no relevant toxicities with no need for dose adjustments. These cases highlight the usefulness of ivosidenib as a targeted therapeutic option for patients with metastatic or recurrent unresectable chondrosarcomas with IDH1 mutation. Long-term efficacy with very good tolerance was achieved.
2026-09-09 | Real-World Efficacy and Safety of Tyrosine Kinase Inhibitors in Patients With Advanced Bone Sarcomas: The TOGBONE Study of the Turkish Oncology Group.
Primary bone sarcomas, including osteosarcoma, chondrosarcoma, and Ewing sarcoma, have poor prognoses, with 5-year overall survival (OS) rates of < 20%-30% in metastatic or recurrent disease. Although tyrosine kinase inhibitors (TKIs) targeting angiogenic and oncogenic pathways have shown efficacy in phase II trials, there is a lack of supporting real-world data. This study aimed to evaluate the efficacy and safety of TKI use in patients with advanced bone sarcomas in a real-world setting. This multicenter, retrospective study included 72 patients with advanced or metastatic osteosarcoma, chondrosarcoma, or Ewing sarcoma who received at least one TKI (regorafenib, pazopanib, sorafenib, or cabozantinib) at multiple centers in Türkiye. Progression-free survival (PFS) and OS were estimated using the Kaplan-Meier method. Adverse events were recorded and graded according to standard criteria. The median age was 28.5 years (range, 17-72 years), and 62.5% (n = 45) of the patients were male. The most frequently used TKIs were regorafenib (n = 31, 43.1%) and pazopanib (n = 22, 30.6%); in the majority of patients (n = 68, 94.4%), TKIs were used as second-line or subsequent treatment. The median PFS for the entire cohort was 4.8 months (95% CI, 3.05 to 6.48), and the median OS was 9.9 months (95% CI, 6.48 to 13.25). In histologic subgroup analyses, the median PFS was 4.4 months for osteosarcoma, 4.8 months for chondrosarcoma, and 5.4 months for Ewing sarcoma. Adverse events were observed in 50% (n = 36) of patients, and 89.2% (n = 58) of these were grade 1-2. Dose reduction was required in 27.8% (n = 20) of patients, and permanent treatment discontinuation due to toxicity was necessary in 6.9% (n = 5) of patients. In this real-world cohort, although TKIs were associated with limited survival outcomes, they had an acceptable safety profile in patients with advanced bone sarcomas. These findings are consistent with the results of phase II studies and suggest that TKIs may be a treatment option for patients who have disease progression after standard therapies.
2026-08-27 | Emerging Treatments in Bone Tumors: Lessons Learned from the ESMO Annual Meeting.
Primary bone sarcomas are rare, heterogeneous malignancies with limited therapeutic options, particularly in metastatic disease where outcomes remain poor. This study synthesizes current literature to evaluate emerging therapeutic strategies and biological determinants of response across osteosarcoma, Ewing sarcoma, and chondrosarcoma, as presented at the ESMO Annual Meeting, 2025. Key approaches reviewed include VEGFR-targeted tyrosine kinase inhibitors (TKIs), DNA damage response (DDR) inhibition, MYC targeting, immune checkpoint inhibitors (ICIs), and surfaceome-directed therapies such as antibody-drug conjugates (ADCs) and chimeric antigen receptor T cell therapy. Across studies, TKIs demonstrated short lasting activity as monotherapy but improved outcomes in some of the studies when combined with ICIs or chemotherapy, reflecting their role in remodeling the tumor microenvironment (TME); importantly, controlled studies with TKI and chemotherapy upfront are ongoing. DDR- and MYC-targeted therapies have shown strong preclinical rationale but limited clinical efficacy, highlighting challenges in translation. Immune-based therapies exhibited variable responses, with dedifferentiated chondrosarcoma (DDCS) emerging as a responsive histotype. Surfaceome-targeting strategies, particularly ADCs, demonstrated promising early clinical activity. Overall, bone sarcoma rarity, tumor heterogeneity, immunosuppressive TME, and lack of predictive factors challenge drug discovery for bone sarcoma patients. These findings underscore the importance of combination strategies and biomarker-driven patient selection and suggest that continued integration of targeted and immunotherapeutic approaches will be critical to improving outcomes in bone sarcoma.
2026-08-14 | Vitamin D decreases tumor cell growth and invasiveness in primary malignant bone tumors in vitro.
Primary malignant bone tumors (PMBTs) are rare neoplasms arising from bone tissue. Limited evidence suggests an association between reduced serum 25(OH) vitamin D3 levels and PMBT incidence. The active metabolite 1,25(OH)2 vitamin D3 (1,25D3) modulates tumor cell proliferation, apoptosis, and migration through interactions with the vitamin D receptor (VDR). This study investigated the effects of 1,25D3 on osteosarcoma (MG-63, SaOS-2), chondrosarcoma (SW-1353), and Ewing's sarcoma (RD-ES) cell lines. Expression of key components of vitamin D metabolism, including 1α-hydroxylase (CYP27B1), 24-hydroxylase (CYP24A1), VDR, protein disulfide-isomerase A3 (PDIA3), and the 1,25D3-responsive bone gamma-carboxyglutamate protein (BGLAP), was analyzed at mRNA and protein levels. VDR nuclear translocation was assessed by Western blotting. Cell viability, cell cycle progression, apoptosis, and migration were evaluated using metabolic assays, flow cytometry, immunocytochemistry, wound healing, and live-cell imaging. Treatment with 1,25D3 upregulated CYP24A1 in osteosarcoma cells, while BGLAP expression was detected in both osteosarcoma and SW-1353 cells. All examined cell lines expressed VDR and PDIA3, and 1,25D3 induced VDR nuclear translocation. MG-63 cell viability decreased by 23%, whereas SW-1353 cell viability decreased by 10%, with no significant changes observed in SaOS-2 and RD-ES cells. In MG-63 cells, 1,25D3 induced a significant accumulation of cells in the G1 phase, consistent with cell-cycle arrest, whereas only minor cell-cycle alterations were observed in the other cell lines. Interestingly, 1,25D3 reduced the proportion of apoptotic osteosarcoma cells by 18-30%. The migration of MG-63 cells was significantly reduced by 1,25D3, and an inhibitory trend, albeit not statistically significant, was also observed in RD-ES cells. In conclusion, 1,25D3 modulated cell viability, apoptosis, and migration in PMBT cell models, with distinct, cell-type-specific responses. These findings suggest a potential role for 1,25D3 in regulating PMBT growth and invasiveness.
2026-08-06 | Chondrosarcoma: A comprehensive review of tumor biology, clinical behavior, and therapeutic advances.
Chondrosarcoma is a heterogeneous group of malignant bone tumors characterized by the production of neoplastic hyaline cartilage. These tumors are often indolent and typically resistant to conventional chemotherapy and radiotherapy, with surgical resection remaining the cornerstone of treatment. However, patients with unresectable or metastatic disease face poor outcomes due to the lack of effective systemic therapies. While advancements in systemic treatments - such as immunotherapy and anti-angiogenic agents - and novel radiation modalities like proton beam therapy and carbon ion therapy have shown some benefit in select cases, their overall efficacy remains limited, underscoring the urgent need for more effective treatment options. Emerging novel therapies are beginning to illuminate potential new treatment pathways. Current efforts are increasingly focused on targeting recurrent genetic alterations and dysregulated signaling pathways, including IDH mutations, DR5-mediated apoptotic pathway, hedgehog signaling, Src kinase pathway, the PI3K-Akt-mTOR axis, histone deacetylation, and angiogenesis. Although these targeted strategies are still under investigation, they show promise in overcoming therapeutic resistance and advancing personalized treatment. This review provides an overview of the clinical characteristics of major chondrosarcoma subtypes, key genetic alterations implicated in tumor pathogenesis, and recent advances in treatment strategies, including surgery, chemotherapy, radiotherapy, immunotherapy, and emerging targeted therapies.
other
2026-07-29 | Dedifferentiated Chondrosarcoma: Current Advances and Future Perspectives.
Dedifferentiated chondrosarcoma (DDCS) remains one of the most aggressive and therapeutically challenging primary bone malignancies. While surgery continues to represent the cornerstone of treatment for localized disease, outcomes remain poor due to high rates of local recurrence and metastatic progression. The role of adjuvant therapies remains less clear. Radiotherapy may be considered in selected patients with unresectable tumors, positive margins, or for symptom palliation, whereas conventional chemotherapy has demonstrated inconsistent and generally limited benefit despite the use of osteosarcoma-based regimens. Recent advances in molecular profiling have improved our understanding of DDCS biology and revealed potential therapeutic opportunities. Recurrent IDH1/2 mutations appear to contribute to tumor progression and dedifferentiation and may represent actionable therapeutic targets. In our practice, comprehensive molecular profiling should be considered whenever feasible, particularly in advanced disease. Emerging evidence suggests that immunotherapy can induce durable responses in a small subset of patients, while early studies combining immune checkpoint inhibitors with antiangiogenic tyrosine kinase inhibitors have demonstrated encouraging response rates that exceed those historically achieved with chemotherapy alone. Although these findings require prospective validation, they support a shift toward biomarker-driven and combination treatment strategies.
2026-05-11 | Case Report: Patient-specific 3D-printed preoperative simulation for tailored resection and reconstruction in complex vertebral tumors: a case of giant recurrent chondrosarcoma at the cervicothoracic junction.
Three-dimensional (3D) printing is a rapidly evolving technology that is transforming various fields and its application in surgery, particularly in spinal procedures, has seen substantial growth in the last 10 years. It enables the production of highly accurate, patient-specific custom implants and anatomical models, enhancing preoperative surgical planning and intraoperative decision-making. This article describes the workflow adopted to produce a 3D-printed model of the cervical column of a patient affected by a recurrent giant cervical chondrosarcoma, focusing on its application in the presurgical resection and reconstruction planning. We present the case of a 67-year-old female patient with recurrent clear cell chondrosarcoma of the cervical spine. After multidisciplinary discussion, a two-stage (posterior and anterior stage) intentional Enneking inappropriate subtotal resection, followed by adjuvant proton beam therapy (PBT), was planned. One week before the second surgical stage, a surgical simulation was performed on a 3D-printed model. For the 3D virtual modeling, contrast-enhanced CT images of the cervicothoracic spine were obtained for the segmentation of the different anatomical structures. A PolyJet J850 Digital Anatomy® (Stratasys, USA) printer was used due to its ability to assign different materials to each structure, closely mimicking real tissue properties. Surgery was completed without complications, with neurological improvement from American Spinal Injury Association (ASIA) C to D. Adequate decompression and stable reconstruction were achieved. Adjuvant PBT was delivered postoperatively. At the 6-month follow-up, imaging demonstrated good local control and early fusion, and the patient was pain-free and functionally independent. The creation of patient-specific, 1:1 scale 3D-printed anatomical models is a crucial tool in the improvement of preoperative planning, providing crucial tactile and visual insights for complex spinal tumor resection and reconstruction, thereby improving surgical precision and safety.
2025-08-30 | Anti-PD-1 antibody plus anlotinib vs. anlotinib alone in patients with refractory chondrosarcoma: A multicenter, non-randomized phase 2 FLAIL-C trial.
There is no standard systemic therapy for unresectable chondrosarcoma. The purpose of this study is to explore the efficacy of combination therapy with an anti-PD-1 antibody and anlotinib in patients with advanced chondrosarcoma. Patients with dedifferentiated or high-grade conventional chondrosarcoma were eligible. Anlotinib was administered at 12 mg orally once a day from day 1 to 14 every 3 weeks in all participants. In the combination treatment arm, patients received an additional anti-PD-1 antibody at 200 mg every 3 weeks. The primary endpoint was the 6-month progression-free survival rate (PFSR). Biomarker analyses for therapeutic effectiveness were conducted. 70 patients (32 with dedifferentiated and 38 with conventional chondrosarcoma) were enrolled in the study. After a medium follow-up of 15.6 months, combination treatment showed significantly improved outcomes compared to anlotinib alone in the entire population, with a higher 6-month PFSR (60.0% versus 31.4%). The PFS-event inverse probability weighting adjusted Cox model evaluation also revealed a significant benefit of combination treatment (hazard ratio = 0.14, 95% confidence interval [CI]: 0.07-0.30, p < 0.001). Patients with dedifferentiated chondrosarcoma benefited the most from the combination treatment, with improvements in objective response rate (33.3% versus 9.1%), 6-month PFSR (57.1% versus 9.1%), median PFS (7.0 months versus 3.8 months), and 1-year overall survival rate (42.9% versus 18.2%). Effector memory T cells were significantly associated with treatment response (p < 0.001). The combination treatment demonstrated promising efficacy in advanced chondrosarcoma, particularly for dedifferentiated cases (ClinicalTrials.gov: NCT05193188). This trial was supported by Chia Tai Tianqing Pharmaceutical Group Co., Ltd.
2025-05-28 | The Double Life of microRNAs in Bone Sarcomas: Oncogenic Drivers and Tumor Suppressors.
Bone sarcomas, including Osteosarcoma, Ewing's sarcoma, and Chondrosarcoma, are rare yet aggressive tumors with high metastatic potential and poor survival outcomes. Despite advances in surgical and chemotherapeutic techniques, these malignancies remain difficult to treat. They often exhibit resistance to conventional therapies and are associated with a limited prognosis for patients. MicroRNAs (miRNAs) have emerged as pivotal regulators of cancer biology, orchestrating crucial processes such as cell proliferation, apoptosis, and metastasis. Their double life as oncogenes or tumor suppressors underscores their significance in the pathogenesis of bone sarcomas. This review examines the multifaceted roles of miRNAs in these malignancies. By elucidating the complex networks affected by miRNA dysregulation, we seek to identify novel avenues for miRNA-based interventions. It is the intention of this work to stimulate future research and clinical strategies that exploit the potential of miRNAs to transform the management and outcomes of bone sarcomas.
2025-03-28 | Expression of Cancer-Testis Antigens MAGE-A1, MAGE-A4, NY-ESO-1 and PRAME in Bone and Soft Tissue Sarcomas: The Experience From a Single Center in China.
Sarcomas are a heterogeneous group of malignancies, low disease-control levels and the limited durability of responses have prompted the exploration of various novel immunotherapeutic approaches. To preliminarily explore the feasibility of cancer vaccines based on cancer testis antigen in the immunotherapy of sarcomas, we investigate the expression of Cancer/Testis Antigens (CTA) MAGE-A4, PRAME, MAGE-A1, KK-LC-1, and NY-ESO-1 in bone and soft tissue sarcomas, with the aim of assessing their potential for use in sarcoma immunotherapy and determining their expression levels in different subtypes. We employed immunohistochemistry and multiplex immunostaining microarrays (MI chips) to assess the expression of MAGE-A4, PRAME, MAGE-A1, KK-LC-1, and NY-ESO-1 in 21 cases of undifferentiated pleomorphic sarcoma (UPS), 26 cases of smooth muscle sarcoma, 28 cases of liposarcoma, 40 cases of osteosarcoma (OS), and 13 cases of chondrosarcoma. MAGE-A1 showed the highest expression in osteosarcoma (32.50%), while it was lower in liposarcoma and undifferentiated pleomorphic sarcoma (10.71% and 10.00%) and undetectable in chondrosarcoma. MAGE-A4 expression was elevated in osteosarcoma and undifferentiated pleomorphic sarcoma (40.00% and 33.00%), but lower in liposarcoma and smooth muscle sarcoma (17.00% and 33.00%). NY-ESO-1 expression was relatively low across all sarcoma subtypes. PRAME expression was highest in undifferentiated pleomorphic sarcoma (47.62%) and low in chondrosarcoma (7.69%). None of the sarcomas expressed KK-LC-1. Additionally, while there was no statistically significant correlation between CTA expression and patient age or gender, some differences related to age and gender were observed. CTA expression in bone and soft tissue sarcomas was correlated with both CTA type and sarcoma subtype, showing relatively high levels of expression in undifferentiated pleomorphic sarcoma (UPS) and osteosarcoma (OS). The poly-expression of MAGE-A4, PRAME, and MAGE-A1 across all subtypes suggests that these antigens may serve as potential targets for sarcoma-specific immunotherapy.
cell therapies
2026-09-25 | [Resection of a chondrosarcoma of the first metacarpal and reconstruction using composite osteosynthesis : Case report and literature review].
Chondrosarcoma is the most common malignant bone tumor of the hand. As conventional chondrosarcomas generally respond poorly to radiotherapy and chemotherapy, surgical resection is crucial. A 76-year-old woman with a chondrosarcoma of the first metacarpal underwent 2‑stage reconstruction using composite osteosynthesis after tumor resection. After 18 months the functional outcome was satisfactory, with no local recurrence, metastases or implant-associated complications. Composite osteosynthesis can represent a function-preserving reconstructive option in selected patients.
2026-09-23 | Restoring thoracic stability with rigid titanium rib reconstruction after radical forequarter and upper chest wall resection for T4 chondrosarcoma.
Extensive anterior chest wall involvement in chondrosarcoma presents major reconstructive challenges following radical oncologic resection. A 55-year-old man with cT4N0M0 chondrosarcoma involving the right shoulder girdle, ribs 1-4, and right upper lobe underwent en bloc extended forequarter amputation, multilevel rib resection, and right upper lobe segmentectomy. Chest wall reconstruction was performed using a titanium-based RibLoc plating system with vascularized soft-tissue flap coverage. Postoperatively, chest wall stability was preserved without paradoxical motion, and radiographs confirmed appropriate implant positioning. The patient was successfully weaned from mechanical ventilation on postoperative day two and transferred from the intensive care unit on day three without major complications. Rigid titanium rib fixation provided effective structural restoration and favorable early postoperative outcomes following extensive anterior chest wall resection for advanced chondrosarcoma.
2026-07-10 | Short-term Functional Outcomes of Shoulder Girdle Limb Salvage in Primary Bone Tumors: A 2-year Follow-up Study.
The shoulder girdle, encompassing the proximal humerus, scapula, lateral third of the clavicle, and surrounding soft tissues, is the third most frequent site for bone tumors. The proximal humerus is the most commonly affected area, followed by the scapula and clavicle. Managing malignant or aggressive benign bone tumors in this region is particularly challenging due to the proximity to vital neurovascular structures and the need to maintain both stability and dexterity in the shoulder. While traditional treatment often involved extensive resections, contemporary limb salvage techniques focus on preserving function while ensuring effective tumor control. This prospective study included 18 patients who underwent various limb salvage procedures at the Department of Orthopaedics, Vydehi Institute of Medical Sciences and Research Centre in Bengaluru between 2012 and 2019, with a minimum follow-up of 2 years. Diagnosis and staging were performed using imaging modalities and core needle biopsies, with musculoskeletal tumors staged according to the Enneking System. Functional outcomes were assessed using the Musculoskeletal Tumor Society Rating Scale (MSTS). Surgical techniques varied and included endoprosthetic reconstruction, arthrodesis with fibular grafting and plating, and plate fixation with primary shortening. Among the 18 patients, the majority (61%) had giant cell tumors, followed by aneurysmal bone cysts (17%), chondrosarcoma (11%), and osteosarcoma (11%). Functional outcomes, as measured by the MSTS, showed a 75% satisfactory rate. The study population was composed of 55.56% males and 44.44% females. Key parameters evaluated included pain, functional activity, hand positioning, dexterity, lifting ability, and emotional acceptance. Limb salvage surgery for primary bone tumors of the shoulder girdle offers favorable functional outcomes and represents a viable alternative to amputation. Modern reconstruction techniques, such as endoprosthetic reconstruction and fibular grafting with plating, achieve satisfactory results in both function and appearance. The MSTS scoring system is an effective tool for assessing postoperative functional status, and there is a growing preference for less aggressive surgical techniques that preserve limb function while ensuring tumor control.
2026-07-02 | Identification of immunopeptides (pHLA) as candidate therapeutic targets in chondrosarcoma
Background Chondrosarcoma (CHS) is the second most common primary malignant bone tumor and remains resistant to conventional therapies, underscoring the need to discover novel therapeutic targets. Cancer/testis antigens (CTAs), a class of tumor-associated proteins, represent attractive antigens for cancer immunotherapies such as adoptive T cell therapy. However, the expression profile of CTAs and their associated targetable immunopeptides presented in the Human Leukocyte Antigen-I context (pHLA) remain unknown in CHS. This study aims to characterize the CTA expression profile according to the tumor immune phenotype and clinical outcomes, and to identify the most relevant pHLA to target in CHS. Method We analyzed the CTA expression profile in tumors from 63 conventional CHS patients and in healthy tissues using GTEx and HPA databases to identify CHS-associated CTAs. Cox proportional hazards models combined with hierarchical clustering were used to correlate CTA expression with the overall survival of patients. The tumor immune phenotype was estimated based on immune gene expression signatures using a deconvolution method and a Pearson correlation coefficient matrix. The CTA-derived pHLA were characterized using immunopeptidomic profiling based on HLA-I immunoprecipitation and mass spectrometry in grade 2 and 3 CHS models. NetMHC was used to predict the binding affinity of identified pHLA to the HLA-A*02:01 and HLA-A*01:01 alleles. Results We identified a poor prognosis CTA signature predominantly associated with a non-inflamed tumor immunophenotype. Immunopeptidomic profiling revealed broad pHLA repertoires, including previously well-characterized CTAs from PRAME, CTAG2 and the MAGE-A family, as well as newly identified candidates with strong predicted HLA-binding affinity from CTAs such as HHIPL2, DBF4, BRIP1, CBX2 and DIAPH3. Conclusion This study provides the first atlas of pHLA in CHS and suggests specific antigenic targets for TCR-T cell therapies and targeted therapies such as antibody-drug conjugates.
2026-06-12 | Robot-Assisted "Postage-Stamp" Vertebrotomy for Spinal Tumor Resection: Case Report.
Background: Achieving adequate oncological margins in tumors involving the thoracic costovertebral junction is technically challenging because of complex regional anatomy and the need to preserve neurological and biomechanical integrity. This case report describes a robot-assisted margin-extension strategy after incomplete resection of a thoracic costovertebral chondrosarcoma. Methods: A 31-year-old man with grade 1 chondrosarcoma of the left sixth rib underwent second-stage surgical radicalization after prior incomplete resection with positive medial margins. Following multidisciplinary discussion, a single-stage posterior procedure was performed, including robot-assisted T4-T8 stabilization with radiolucent CFR-PEEK instrumentation and robot-assisted sagittal vertebral osteotomy ("Postage-Stamp Osteotomy") of T6 to achieve en bloc removal of the involved costovertebral segment. Results: The osteotomy was executed using a pedicle-referenced robotic trajectory workflow with sequential navigated drilling and controlled completion with a navigated osteotome. Total operative time was 379 min, with estimated blood loss of 800 mL. No major intraoperative neurovascular complications occurred. Histopathology confirmed negative margins. The patient was mobilized on postoperative day 1 and discharged on postoperative day 6 without new neurological deficits. Radiological follow-up at 3 months showed no recurrence, while clinical follow-up at 5 months demonstrated full return to baseline activities. Conclusions: This report describes a technically feasible robot-assisted margin-extension strategy in a highly selected thoracic spinal oncology scenario. Although long-term oncological conclusions cannot be drawn from a single case, tailored technology-enabled margin-oriented approaches may represent a case-specific option in carefully selected patients.
antibodies
2026-09-24 | Radiological and multidisciplinary management of extraskeletal myxoid chondrosarcoma: A case report.
Extraskeletal myxoid chondrosarcoma (EMC), a very rare subtype of soft tissue sarcoma (STS), distinguished by its histopathological profile, has unique clinical prognosis of potential for late local recurrences and distant metastases due to its molecular alterations. The infrequent occurrence of EMC, coupled with its radiological characteristics that often overlap with those of other myxoid-rich neoplasms, may impose considerable diagnostic difficulties for clinicians. A 43-year-old male who presented with a progressively enlarging mass in the distal right thigh and knee region since six-month prior. Initial imaging studies, including computed tomography (CT) and non-contrast magnetic resonance imaging (MRI), suggested a diagnosis of liposarcoma. However, further evaluation including a comprehensive contrast-enhanced MRI revealed a large multilobulated, well-defined soft tissue mass at the distal anteromedial femur. The lesion demonstrated isointense to hypointense signal on T1-weighted sequence and hyperintensity on T2-weighted/STIR sequences reflecting myxoid matrix, and prominent peripheral and septal enhancement post-gadolinium administration. The mass also exhibited local bone erosion and infiltration into surrounding musculature and the knee joint. A core needle biopsy followed by immunohistochemical profiling was consistent with a diagnosis of EMC. Following multidisciplinary consultation, the patient underwent successful limb salvage surgery, involving wide excision of the tumor and reconstruction using prosthesis. This case underscores the critical role of advanced imaging techniques in characterizing soft tissue masses and suggesting EMC in the differential diagnosis prior to histopathological confirmation. Moreover, multidisciplinary approach for the effective management of this rare sarcoma, including complex surgical reconstruction to preserve limb function is essential.
2026-09-18 | Emerging therapeutic strategies and clinical development of investigational agents for conventional chondrosarcoma.
Conventional chondrosarcoma (cCS) is a malignant bone tumor with intrinsic resistance to cytotoxic therapy. Advances in genomic profiling have revealed recurrent alterations that are redefining the therapeutic landscape for these tumors. Literature searches of PubMed and ClinicalTrials.gov for the terms conventional chondrosarcoma, systemic therapy, targeted therapy, immunotherapy, chemotherapy, molecular targets, and clinical trial through June 2026 were performed. Clinical and translational developments regarding treatment of cCS were summarized, emphasizing the following emerging targeted and immunotherapeutic agents: isocitrate dehydrogenase 1 and 2 (IDH1/2) inhibitors, DR5 agonists, methylthioadenosine phosphorylase (MTAP)-deficient PRMT5 inhibitors, TP53 reactivators, histone deacetylase and BCL-2 modulators, immunotherapy approaches, multi-targeted tyrosine kinase inhibitors (TKIs) and antibody-drug conjugates. Multi-targeted TKIs remain the most commonly used systemic therapies for advanced cCS; however, recent advances in DR5 agonism and IDH1 inhibition provide proof of concept that biologically informed therapeutic strategies can achieve meaningful clinical benefit. Continued progress will require comprehensive molecular profiling, rational combination strategies, and innovative clinical trial designs to accelerate drug development in this rare malignancy.
2026-08-31 | Primary Mesenchymal Chondrosarcoma of the Spine Masquerading as Potts Spine: A Case Report
Background: Primary mesenchymal chondrosarcoma is a rare malignant neoplasm originating from bone and soft tissue commonly involving maxilla and mandible, but can also involve ribs, pelvis, femur, and spine. Case report: 16 years-old-female presented with weakness and paresthesia of lower limbs since 15 days with difficulty in walking. CT scan showed pre and paravertebral soft tissue density lesion extending from D8 to D12 vertebral body levels. MRI showed heterogenous lesion with hypo and hyperintense areas in T1W1 and hyperintense on T2. Tumor is excised and on histopathological examination revealed the features of mesenchymal chondrosarcoma. Conclusion: Mesenchymal chondrosarcoma is a rare malignant mesenchymal tumor located in bone, intracranial sites or soft tissue with non-specific findings on imaging. Histopathology and additional immunohistochemistry is gold standard for diagnosis.
2026-06-25 | Chondrosarcoma of the Mandible: A Clinicopathological Case Report based Insight
Chondrosarcoma is a malignant mesenchymal tumour characterized by the production of cartilaginous matrix. Its occurrence within the maxillofacial region is uncommon and frequently presents a diagnostic dilemma because its clinical and radiographic features can resemble other primary bone malignancies. A 70-year-old male presented with a gradually enlarging and painful swelling involving the left side of the mandible over a period of approximately six months. Clinical examination revealed a diffuse extraoral swelling along with an intraoral exophytic and ulcerated lesion in the posterior alveolar region. Imaging studies demonstrated irregular radiopaque areas associated with cortical destruction and a characteristic sunburst-type periosteal reaction. Histopathological examination showed atypical chondrocytes embedded within a chondroid matrix, along with areas of calcification and cellular pleomorphism, confirming the diagnosis of chondrosarcoma. This case highlights the aggressive nature of mandibular chondrosarcoma and emphasizes the importance of correlating clinical, radiographic, and histopathological findings to arrive at a definitive diagnosis and guide appropriate treatment.
2026-05-27 | Pembrolizumab for MSI-high/TMB-high bone and soft tissue sarcomas: a retrospective single-institution study.
Bone and soft tissue sarcomas are rare, heterogeneous malignancies with limited treatment options for advanced stages. Pembrolizumab, an anti-PD-1 immune checkpoint inhibitor (ICI), is approved for tumors exhibiting high microsatellite instability (MSI-high) or high tumor mutation burden (TMB-high); however, its efficacy remains unclear. We reviewed records of 40 patients with advanced/recurrent sarcoma who underwent genomic profiling. MSI status and TMB were assessed using the NCC Oncopanel® or FoundationOne®. Patients identified as MSI-high and/or TMB-high received pembrolizumab (200 mg/body weight every 3 weeks). Clinical outcomes were evaluated using RECIST v1.1 and adverse events graded per CTCAE v5.0. Three patients (7.5%) were MSI-high and/or TMB-high: one each with undifferentiated pleomorphic bone sarcoma, leiomyosarcoma, and dedifferentiated chondrosarcoma. All had metastatic disease and received chemotherapy. Pembrolizumab was administered for 5-25 cycles for 5-18 months. Two patients achieved a partial response, and one had stable disease, yielding an objective response rate of 67% and a mean response duration of 12.7 months. No complete response or disease progression occurred. The adverse events were limited to grade 2 thyroid dysfunction and skin toxicity. No grade ≥3 events or treatment discontinuations occurred. Pembrolizumab demonstrated promising efficacy and tolerability in MSI-high/TMB-high sarcomas. These findings support biomarker-driven immunotherapy in patients with sarcomas. Comprehensive genomic profiling should be considered in refractory cases. Further studies are warranted to validate these results and explore additional biomarkers.
proteins
2026-09-07 | A logic-gated synthetic circuit coupled with microenvironmental reprogramming overcomes immune exclusion in antigen-poor chondrosarcoma.
Chondrosarcoma remains refractory to immunotherapy due to a paucity of targetable antigens and a suppressive tumor microenvironment (TME). We developed a modular strategy integrating tumor-intrinsic genetic logic with rational TME reprogramming to overcome these barriers. Using a 57,715-element massively parallel reporter assay, we identified chondrosarcoma-selective synthetic promoters and engineered a Boolean AND-gate circuit that ensures high-fidelity expression of a surface T cell engager exclusively within malignant cells. To further address the immunosuppressive TME, we identified macrophage migration inhibitory factor (MIF) as a target for blockade. Single-cell RNA sequencing in human PBMC-reconstituted chondrosarcoma xenograft models revealed that the circuit initiates T cell activation, while MIF blockade prevents exhaustion by promoting proliferative T cells and M1-polarized macrophages. Local administration induced a systemic abscopal effect, regressing distant, untreated tumors. Collectively, these findings provide a programmable blueprint for converting immunologically cold, antigen-poor malignancies into systemically immune-responsive states.
2026-05-21 | Analysis code and notebooks for: Identification of immunopeptides (pHLA) as candidate therapeutic targets in chondrosarcoma
This repository contains the analysis code and notebooks associated with the study on cancer/testis antigens (CTAs) and immunopeptides (pHLA) in chondrosarcoma (CHS). It includes transcriptomic analyses of CTA expression profiles using public databases (GTEx, HPA, E-MTAB-7264), survival analyses (Cox models and hierarchical clustering), tumor immune phenotype deconvolution, and immunopeptidomic data processing. Key findings include the identification of a poor prognosis CTA signature associated with low immune cell infiltration, and the characterization of pHLA repertoires in CHS cell lines, with the identification of novel candidate therapeutic targets.
2026-03-14 | Vasculogenic Mimicry: A Potential Therapeutic Target for Chondrosarcoma Therapy.
Chondrosarcomas (ChSs) are mesenchymal chemo- and radiation-resistant tumors, representing the second most frequently diagnosed bone sarcoma after osteosarcoma and 20% of all bone sarcomas. Most of ChS patients have a good prognosis after complete surgical resection. Conversely, patients with inoperable disease, due to the tumor location or metastatic dissemination, represent a great clinical challenge due to the lack of effective therapeutic options. In this study, to the best of our knowledge, we document, for the first time in human ChS tissues, the existence of CD-31- and Podoplanin-negative vascular-like channels containing red blood cells, allowing us to hypothesize the occurrence of vasculogenic mimicry (VM) in ChSs. By using patient-derived ChS cells and a stabilized ChS cell line, we demonstrate that ChS cells are able to form in vitro tubules apparently similar to those formed by endothelial cells. Further characterization of these vessels revealed the pivotal role of the Urokinase Plasminogen Activator Receptor (uPAR) in mediating the capability of ChS cells to form VM. Finally, we provide evidence that, unlike bevacizumab, which did not exert any effect, the uPAR-derived antiangiogenic peptide RI-3 behaves as a potent inhibitor of VM.
2025-07-05 | Pediatric mesenchymal chondrosarcoma with archetypal histology in the absence of HEY1::NCOA2 gene fusion: a molecular-morphological paradox.
Mesenchymal chondrosarcoma (MC) is a rare, aggressive malignancy characterized by a distinctive biphasic histological pattern and frequently associated with the HEY1::NCOA2 fusion gene. We report a case of a 17-year-old male presenting with a mandibular mass initially suspected to be a benign chondro-osseous lesion. Following segmental mandibulectomy, histopathological examination revealed classical features of MC, including undifferentiated small round to spindle cells arranged around staghorn vasculature and islands of mature hyaline cartilage. Immunohistochemistry showed strong S100 positivity in the cartilaginous component and diffuse NKX3.1 expression in the undifferentiated areas. However, molecular analysis via next-generation sequencing did not detect HEY1::NCOA2 fusion, raising the possibility of a fusion-negative variant. This case highlights the potential for molecular heterogeneity in MC and emphasizes that, in the presence of unequivocal morphology, absence of canonical gene fusions should not preclude the diagnosis. It reinforces the enduring role of histopathology and the need for expanded molecular profiling in such diagnostically challenging cases.
2025-04-15 | Protein Phosphatase 1 Regulatory Subunit 3C integrates cholesterol metabolism and isocitrate dehydrogenase in chondrocytes and neoplasia.
Enchondromas are common bone tumors composed of chondrocytes originating from growth plate cells which can progress to malignant chondrosarcoma. Mutations in the genes encoding isocitrate dehydrogenase (IDH1 and IDH2) are identified in a large proportion of these tumors. IDH enzymes convert isocitrate to alpha-ketoglutarate (α-KG), an essential component of the citric acid cycle. While mutant IDH enzymes produce 2-hydroxyglutarate, which has epigenetic effects important in tumor initiation, cell maintenance and growth rely on additional factors. Prior work shows that intracellular cholesterol and glycogen are upregulated in mutant IDH chondrocytes. Here, we show that Protein Phosphatase 1 Regulatory Subunit 3C (PPP1R3C, previously termed Protein Targeting to Glycogen or PTG) is highly expressed in chondrocytes harboring a mutant IDH. Furthermore, Sterol Regulatory Element-Binding Proteins (SREBPs), transcriptional regulators of sterol biosynthesis, regulate PPP1R3C expression. We found that PPP1R3C regulates glycolysis and glycolytic capacity in chondrocytes. Depletion of PPP1R3C in mouse chondrocytes in vivo suppresses the neoplastic phenotype. The growth plate phenotype associated with the genetic inhibition of cholesterol biosynthesis is partially rescued by PPP1R3C overexpression. Taken together, our data show that PPP1R3C integrates cholesterol metabolism and isocitrate dehydrogenase in growth plate and neoplastic chondrocyte metabolism by regulating intracellular glycogen levels.
small molecules
2026-09-11 | Targeting IDH1: Long-Term Efficacy and Tolerance of Ivosidenib in Advanced Chondrosarcoma.
Around 20% of primary bone malignancies are chondrosarcomas. Advanced or recurrent conventional chondrosarcomas are typically resistant to chemotherapy and radiotherapy and overall survival remains poor, highlighting the need for other therapeutic options. We present two cases of metastatic chondrosarcoma with isocitrate dehydrogenase 1 (IDH1) mutation treated with ivosidenib. In case 1, a 70-year-old woman with recurrent metastatic grade 2 chondrosarcoma achieved well-tolerated, durable stable disease for 12 months in second line (no response to conventional first line chemotherapy). In case 2, a 63-year-old man with recurrence of grade 2 chondrosarcoma maintained stable disease for 16 months on ivosidenib first line. In both cases, there were no relevant toxicities with no need for dose adjustments. These cases highlight the usefulness of ivosidenib as a targeted therapeutic option for patients with metastatic or recurrent unresectable chondrosarcomas with IDH1 mutation. Long-term efficacy with very good tolerance was achieved.
2026-09-09 | Real-World Efficacy and Safety of Tyrosine Kinase Inhibitors in Patients With Advanced Bone Sarcomas: The TOGBONE Study of the Turkish Oncology Group.
Primary bone sarcomas, including osteosarcoma, chondrosarcoma, and Ewing sarcoma, have poor prognoses, with 5-year overall survival (OS) rates of < 20%-30% in metastatic or recurrent disease. Although tyrosine kinase inhibitors (TKIs) targeting angiogenic and oncogenic pathways have shown efficacy in phase II trials, there is a lack of supporting real-world data. This study aimed to evaluate the efficacy and safety of TKI use in patients with advanced bone sarcomas in a real-world setting. This multicenter, retrospective study included 72 patients with advanced or metastatic osteosarcoma, chondrosarcoma, or Ewing sarcoma who received at least one TKI (regorafenib, pazopanib, sorafenib, or cabozantinib) at multiple centers in Türkiye. Progression-free survival (PFS) and OS were estimated using the Kaplan-Meier method. Adverse events were recorded and graded according to standard criteria. The median age was 28.5 years (range, 17-72 years), and 62.5% (n = 45) of the patients were male. The most frequently used TKIs were regorafenib (n = 31, 43.1%) and pazopanib (n = 22, 30.6%); in the majority of patients (n = 68, 94.4%), TKIs were used as second-line or subsequent treatment. The median PFS for the entire cohort was 4.8 months (95% CI, 3.05 to 6.48), and the median OS was 9.9 months (95% CI, 6.48 to 13.25). In histologic subgroup analyses, the median PFS was 4.4 months for osteosarcoma, 4.8 months for chondrosarcoma, and 5.4 months for Ewing sarcoma. Adverse events were observed in 50% (n = 36) of patients, and 89.2% (n = 58) of these were grade 1-2. Dose reduction was required in 27.8% (n = 20) of patients, and permanent treatment discontinuation due to toxicity was necessary in 6.9% (n = 5) of patients. In this real-world cohort, although TKIs were associated with limited survival outcomes, they had an acceptable safety profile in patients with advanced bone sarcomas. These findings are consistent with the results of phase II studies and suggest that TKIs may be a treatment option for patients who have disease progression after standard therapies.
2026-08-27 | Emerging Treatments in Bone Tumors: Lessons Learned from the ESMO Annual Meeting.
Primary bone sarcomas are rare, heterogeneous malignancies with limited therapeutic options, particularly in metastatic disease where outcomes remain poor. This study synthesizes current literature to evaluate emerging therapeutic strategies and biological determinants of response across osteosarcoma, Ewing sarcoma, and chondrosarcoma, as presented at the ESMO Annual Meeting, 2025. Key approaches reviewed include VEGFR-targeted tyrosine kinase inhibitors (TKIs), DNA damage response (DDR) inhibition, MYC targeting, immune checkpoint inhibitors (ICIs), and surfaceome-directed therapies such as antibody-drug conjugates (ADCs) and chimeric antigen receptor T cell therapy. Across studies, TKIs demonstrated short lasting activity as monotherapy but improved outcomes in some of the studies when combined with ICIs or chemotherapy, reflecting their role in remodeling the tumor microenvironment (TME); importantly, controlled studies with TKI and chemotherapy upfront are ongoing. DDR- and MYC-targeted therapies have shown strong preclinical rationale but limited clinical efficacy, highlighting challenges in translation. Immune-based therapies exhibited variable responses, with dedifferentiated chondrosarcoma (DDCS) emerging as a responsive histotype. Surfaceome-targeting strategies, particularly ADCs, demonstrated promising early clinical activity. Overall, bone sarcoma rarity, tumor heterogeneity, immunosuppressive TME, and lack of predictive factors challenge drug discovery for bone sarcoma patients. These findings underscore the importance of combination strategies and biomarker-driven patient selection and suggest that continued integration of targeted and immunotherapeutic approaches will be critical to improving outcomes in bone sarcoma.
2026-08-14 | Vitamin D decreases tumor cell growth and invasiveness in primary malignant bone tumors in vitro.
Primary malignant bone tumors (PMBTs) are rare neoplasms arising from bone tissue. Limited evidence suggests an association between reduced serum 25(OH) vitamin D3 levels and PMBT incidence. The active metabolite 1,25(OH)2 vitamin D3 (1,25D3) modulates tumor cell proliferation, apoptosis, and migration through interactions with the vitamin D receptor (VDR). This study investigated the effects of 1,25D3 on osteosarcoma (MG-63, SaOS-2), chondrosarcoma (SW-1353), and Ewing's sarcoma (RD-ES) cell lines. Expression of key components of vitamin D metabolism, including 1α-hydroxylase (CYP27B1), 24-hydroxylase (CYP24A1), VDR, protein disulfide-isomerase A3 (PDIA3), and the 1,25D3-responsive bone gamma-carboxyglutamate protein (BGLAP), was analyzed at mRNA and protein levels. VDR nuclear translocation was assessed by Western blotting. Cell viability, cell cycle progression, apoptosis, and migration were evaluated using metabolic assays, flow cytometry, immunocytochemistry, wound healing, and live-cell imaging. Treatment with 1,25D3 upregulated CYP24A1 in osteosarcoma cells, while BGLAP expression was detected in both osteosarcoma and SW-1353 cells. All examined cell lines expressed VDR and PDIA3, and 1,25D3 induced VDR nuclear translocation. MG-63 cell viability decreased by 23%, whereas SW-1353 cell viability decreased by 10%, with no significant changes observed in SaOS-2 and RD-ES cells. In MG-63 cells, 1,25D3 induced a significant accumulation of cells in the G1 phase, consistent with cell-cycle arrest, whereas only minor cell-cycle alterations were observed in the other cell lines. Interestingly, 1,25D3 reduced the proportion of apoptotic osteosarcoma cells by 18-30%. The migration of MG-63 cells was significantly reduced by 1,25D3, and an inhibitory trend, albeit not statistically significant, was also observed in RD-ES cells. In conclusion, 1,25D3 modulated cell viability, apoptosis, and migration in PMBT cell models, with distinct, cell-type-specific responses. These findings suggest a potential role for 1,25D3 in regulating PMBT growth and invasiveness.
2026-08-06 | Chondrosarcoma: A comprehensive review of tumor biology, clinical behavior, and therapeutic advances.
Chondrosarcoma is a heterogeneous group of malignant bone tumors characterized by the production of neoplastic hyaline cartilage. These tumors are often indolent and typically resistant to conventional chemotherapy and radiotherapy, with surgical resection remaining the cornerstone of treatment. However, patients with unresectable or metastatic disease face poor outcomes due to the lack of effective systemic therapies. While advancements in systemic treatments - such as immunotherapy and anti-angiogenic agents - and novel radiation modalities like proton beam therapy and carbon ion therapy have shown some benefit in select cases, their overall efficacy remains limited, underscoring the urgent need for more effective treatment options. Emerging novel therapies are beginning to illuminate potential new treatment pathways. Current efforts are increasingly focused on targeting recurrent genetic alterations and dysregulated signaling pathways, including IDH mutations, DR5-mediated apoptotic pathway, hedgehog signaling, Src kinase pathway, the PI3K-Akt-mTOR axis, histone deacetylation, and angiogenesis. Although these targeted strategies are still under investigation, they show promise in overcoming therapeutic resistance and advancing personalized treatment. This review provides an overview of the clinical characteristics of major chondrosarcoma subtypes, key genetic alterations implicated in tumor pathogenesis, and recent advances in treatment strategies, including surgery, chemotherapy, radiotherapy, immunotherapy, and emerging targeted therapies.
other
2026-07-29 | Dedifferentiated Chondrosarcoma: Current Advances and Future Perspectives.
Dedifferentiated chondrosarcoma (DDCS) remains one of the most aggressive and therapeutically challenging primary bone malignancies. While surgery continues to represent the cornerstone of treatment for localized disease, outcomes remain poor due to high rates of local recurrence and metastatic progression. The role of adjuvant therapies remains less clear. Radiotherapy may be considered in selected patients with unresectable tumors, positive margins, or for symptom palliation, whereas conventional chemotherapy has demonstrated inconsistent and generally limited benefit despite the use of osteosarcoma-based regimens. Recent advances in molecular profiling have improved our understanding of DDCS biology and revealed potential therapeutic opportunities. Recurrent IDH1/2 mutations appear to contribute to tumor progression and dedifferentiation and may represent actionable therapeutic targets. In our practice, comprehensive molecular profiling should be considered whenever feasible, particularly in advanced disease. Emerging evidence suggests that immunotherapy can induce durable responses in a small subset of patients, while early studies combining immune checkpoint inhibitors with antiangiogenic tyrosine kinase inhibitors have demonstrated encouraging response rates that exceed those historically achieved with chemotherapy alone. Although these findings require prospective validation, they support a shift toward biomarker-driven and combination treatment strategies.
2026-05-11 | Case Report: Patient-specific 3D-printed preoperative simulation for tailored resection and reconstruction in complex vertebral tumors: a case of giant recurrent chondrosarcoma at the cervicothoracic junction.
Three-dimensional (3D) printing is a rapidly evolving technology that is transforming various fields and its application in surgery, particularly in spinal procedures, has seen substantial growth in the last 10 years. It enables the production of highly accurate, patient-specific custom implants and anatomical models, enhancing preoperative surgical planning and intraoperative decision-making. This article describes the workflow adopted to produce a 3D-printed model of the cervical column of a patient affected by a recurrent giant cervical chondrosarcoma, focusing on its application in the presurgical resection and reconstruction planning. We present the case of a 67-year-old female patient with recurrent clear cell chondrosarcoma of the cervical spine. After multidisciplinary discussion, a two-stage (posterior and anterior stage) intentional Enneking inappropriate subtotal resection, followed by adjuvant proton beam therapy (PBT), was planned. One week before the second surgical stage, a surgical simulation was performed on a 3D-printed model. For the 3D virtual modeling, contrast-enhanced CT images of the cervicothoracic spine were obtained for the segmentation of the different anatomical structures. A PolyJet J850 Digital Anatomy® (Stratasys, USA) printer was used due to its ability to assign different materials to each structure, closely mimicking real tissue properties. Surgery was completed without complications, with neurological improvement from American Spinal Injury Association (ASIA) C to D. Adequate decompression and stable reconstruction were achieved. Adjuvant PBT was delivered postoperatively. At the 6-month follow-up, imaging demonstrated good local control and early fusion, and the patient was pain-free and functionally independent. The creation of patient-specific, 1:1 scale 3D-printed anatomical models is a crucial tool in the improvement of preoperative planning, providing crucial tactile and visual insights for complex spinal tumor resection and reconstruction, thereby improving surgical precision and safety.
2025-08-30 | Anti-PD-1 antibody plus anlotinib vs. anlotinib alone in patients with refractory chondrosarcoma: A multicenter, non-randomized phase 2 FLAIL-C trial.
There is no standard systemic therapy for unresectable chondrosarcoma. The purpose of this study is to explore the efficacy of combination therapy with an anti-PD-1 antibody and anlotinib in patients with advanced chondrosarcoma. Patients with dedifferentiated or high-grade conventional chondrosarcoma were eligible. Anlotinib was administered at 12 mg orally once a day from day 1 to 14 every 3 weeks in all participants. In the combination treatment arm, patients received an additional anti-PD-1 antibody at 200 mg every 3 weeks. The primary endpoint was the 6-month progression-free survival rate (PFSR). Biomarker analyses for therapeutic effectiveness were conducted. 70 patients (32 with dedifferentiated and 38 with conventional chondrosarcoma) were enrolled in the study. After a medium follow-up of 15.6 months, combination treatment showed significantly improved outcomes compared to anlotinib alone in the entire population, with a higher 6-month PFSR (60.0% versus 31.4%). The PFS-event inverse probability weighting adjusted Cox model evaluation also revealed a significant benefit of combination treatment (hazard ratio = 0.14, 95% confidence interval [CI]: 0.07-0.30, p < 0.001). Patients with dedifferentiated chondrosarcoma benefited the most from the combination treatment, with improvements in objective response rate (33.3% versus 9.1%), 6-month PFSR (57.1% versus 9.1%), median PFS (7.0 months versus 3.8 months), and 1-year overall survival rate (42.9% versus 18.2%). Effector memory T cells were significantly associated with treatment response (p < 0.001). The combination treatment demonstrated promising efficacy in advanced chondrosarcoma, particularly for dedifferentiated cases (ClinicalTrials.gov: NCT05193188). This trial was supported by Chia Tai Tianqing Pharmaceutical Group Co., Ltd.
2025-05-28 | The Double Life of microRNAs in Bone Sarcomas: Oncogenic Drivers and Tumor Suppressors.
Bone sarcomas, including Osteosarcoma, Ewing's sarcoma, and Chondrosarcoma, are rare yet aggressive tumors with high metastatic potential and poor survival outcomes. Despite advances in surgical and chemotherapeutic techniques, these malignancies remain difficult to treat. They often exhibit resistance to conventional therapies and are associated with a limited prognosis for patients. MicroRNAs (miRNAs) have emerged as pivotal regulators of cancer biology, orchestrating crucial processes such as cell proliferation, apoptosis, and metastasis. Their double life as oncogenes or tumor suppressors underscores their significance in the pathogenesis of bone sarcomas. This review examines the multifaceted roles of miRNAs in these malignancies. By elucidating the complex networks affected by miRNA dysregulation, we seek to identify novel avenues for miRNA-based interventions. It is the intention of this work to stimulate future research and clinical strategies that exploit the potential of miRNAs to transform the management and outcomes of bone sarcomas.
2025-03-28 | Expression of Cancer-Testis Antigens MAGE-A1, MAGE-A4, NY-ESO-1 and PRAME in Bone and Soft Tissue Sarcomas: The Experience From a Single Center in China.
Sarcomas are a heterogeneous group of malignancies, low disease-control levels and the limited durability of responses have prompted the exploration of various novel immunotherapeutic approaches. To preliminarily explore the feasibility of cancer vaccines based on cancer testis antigen in the immunotherapy of sarcomas, we investigate the expression of Cancer/Testis Antigens (CTA) MAGE-A4, PRAME, MAGE-A1, KK-LC-1, and NY-ESO-1 in bone and soft tissue sarcomas, with the aim of assessing their potential for use in sarcoma immunotherapy and determining their expression levels in different subtypes. We employed immunohistochemistry and multiplex immunostaining microarrays (MI chips) to assess the expression of MAGE-A4, PRAME, MAGE-A1, KK-LC-1, and NY-ESO-1 in 21 cases of undifferentiated pleomorphic sarcoma (UPS), 26 cases of smooth muscle sarcoma, 28 cases of liposarcoma, 40 cases of osteosarcoma (OS), and 13 cases of chondrosarcoma. MAGE-A1 showed the highest expression in osteosarcoma (32.50%), while it was lower in liposarcoma and undifferentiated pleomorphic sarcoma (10.71% and 10.00%) and undetectable in chondrosarcoma. MAGE-A4 expression was elevated in osteosarcoma and undifferentiated pleomorphic sarcoma (40.00% and 33.00%), but lower in liposarcoma and smooth muscle sarcoma (17.00% and 33.00%). NY-ESO-1 expression was relatively low across all sarcoma subtypes. PRAME expression was highest in undifferentiated pleomorphic sarcoma (47.62%) and low in chondrosarcoma (7.69%). None of the sarcomas expressed KK-LC-1. Additionally, while there was no statistically significant correlation between CTA expression and patient age or gender, some differences related to age and gender were observed. CTA expression in bone and soft tissue sarcomas was correlated with both CTA type and sarcoma subtype, showing relatively high levels of expression in undifferentiated pleomorphic sarcoma (UPS) and osteosarcoma (OS). The poly-expression of MAGE-A4, PRAME, and MAGE-A1 across all subtypes suggests that these antigens may serve as potential targets for sarcoma-specific immunotherapy.
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Drug Discovery Landscape
6 orphan drug designations for Chondrosarcoma.
6 orphan drug designations for Chondrosarcoma.
Drug | Therapy type | Orphan designation | Clinical trial status | Sponsor |
|---|---|---|---|---|
Ivosidenib | small molecules | EMA | Les Laboratoires Servier | |
ivosidenib | small molecules | FDA | Servier Pharmaceuticals | |
Humanised IgG1 tetravalent monoclonal antibody against death receptor 5 | antibodies | EMA | TMC Pharma (EU) Limited | |
ozekibart | antibodies | FDA | Inhibrx, Inc. | |
Patidegib [IPI-926] | small molecules | EMA | Voisin Consulting Life Sciences | |
oral potent and selective antagonist | small molecules | FDA | Infinity Pharmaceuticals, Inc. |
“Terminated” – programs with discontinued clinical trials, or with no updates reported for 5 years after completion or 12 years after the last trial started.
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