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Background Trabectedin is reported to be particularly effective against translocation-related sarcoma. Recently, a randomized phase 2 study in patients with translocation-related sarcomas unresponsive or intolerable to standard chemotherapy was conducted, which showed clinical benefit of trabectedin compared with best supportive care (BSC). Extraskeletal myxoid chondrosarcoma (EMCS) and Mesenchymal chondrosarcoma (MCS) are very rare malignant soft tissue sarcomas, and are associated with translocations resulting in fusion genes. In addition, the previous in vivo data showed that trabectedin affect tumor necrosis and reduction in vascularization in a xenograft model of a human high-grade chondrosarcoma. The aim of the present analysis was to clarify the efficacy of trabectedin for EMCS and MCS subjects in the randomized phase 2 study. Methods Five subjects with EMCS and MCS received trabectedin treatment in the randomized phase 2 study. Three MCS subjects were allocated to the BSC group. Objective response and progression-free survival (PFS) were assessed according to the Response Evaluation Criteria in Solid Tumors (RECIST) version 1.1 by central radiology imaging review. Results The median follow-up time of the randomized phase 2 study was 22.7 months, and one subject with MCS was still receiving trabectedin treatment at the final data cutoff. The median PFS was 12.5 months (95 % CI: 7.4–not reached) in the trabectedin group, while 1.0 months (95 % CI: 0.3–1.0 months) in MCS subjects of the BSC group. The six-month progression-free rate was 100 % in the trabectedin group. One subject with MCS showed partial response, and the others in the trabectedin group showed stable disease. Overall survival of EMCS and MCS subjects was 26.4 months (range, 10.4–26.4 months) in the trabectedin group. At the final data cutoff, two of five subjects were still alive. Conclusions This sub-analysis shows that trabectedin is effective for patients with EMCS and MCS compared with BSC. The efficacy results were better than previously reported data of TRS. These facts suggest that trabectedin become an important choice of treatment for patients with advanced EMCS or MCS who failed or were intolerable to standard chemotherapy. Trial registration The randomized phase 2 study is registered with the Japan Pharmaceutical Information Center, number JapicCTI-121850 (May 31, 2012).

The research retrospectively analyzed cases of spinal chordoma and chondrosarcoma involving patients who received treatment at the two hospitals between 2001 and 2023. Among the 48 patients studied (39 chordoma and 9 chondrosarcoma cases), the average age was 53.9 ± 15.8 years, with a range of 17 to 86 years. Out of these patients, 43 underwent excision surgery and were categorized based on tumor margin into negative (R0) or microscopically positive (R1) margin ( n  = 14) and macroscopically positive (R2) margin ( n  = 29) groups. The mean overall survival (OS) for R0/R1 and R2 groups was 156.5 ± 19.3 and 79.2 ± 11.9 months, respectively ( p value = 0.012). The mean progression-free survival (PFS) for R0/R1 and R2 was 112.9 ± 24.4 and 25.5 ± 5.5 months ( p value < 0.001). The study showed that regardless of whether patients in the R0/R1 or R2 groups received radiation therapy (RT) or not, there was no significant improvement in OS or PFS. Specifically, the OS and PFS for the RT only group were 75.9 ± 16.6 and 73.3 ± 18.0 months. In conclusion, the recommended treatment approach for spinal chordoma and chondrosarcoma remains en bloc resection surgery with an appropriate margin. Patients who are unsuitable for or decline surgery may find a beneficial disease control rate with traditional external beam photon/proton therapy.

Introduction Spinal chondrosarcoma exhibits higher invasiveness and a worse prognosis compared to chondrosarcoma in the extremities. The prognosis and therapeutic plan vary greatly among different pathological subtypes of chondrosarcoma. This study aimed to analyze the differences in clinical characteristics, molecular features, therapeutic effects, and prognostic factors among the subtypes of chondrosarcoma in the spine. Methods A retrospective review was conducted on 205 patients with spinal chondrosarcoma. The clinical features and immunohistochemical (IHC) markers were compared among the pathological subtypes of chondrosarcoma grade 1, grade 2, grade 3, mesenchymal chondrosarcoma (MCS), dedifferentiated chondrosarcoma (DCS), and clear cell chondrosarcoma (CCCS). Chondrosarcoma grade 1/2/3 are collectively referred to as conventional chondrosarcoma (CCS) for multivariate survival analysis. Univariate and multivariate analyses were performed to investigate independent prognostic factors for overall survival (OS) and recurrence-free survival (RFS) in patients with spinal chondrosarcoma. Furthermore, independent prognostic factors for OS and RFS were identified in CCS and MCS. Results MCS patients were younger than the other subtypes. Patients with chondrosarcoma grade 1/2 had better OS than those with chondrosarcoma grade 3, MCS and DCS, while only chondrosarcoma grade 1 patients showed better RFS than chondrosarcoma grade 2/3, MCS and DCS patients. Ki-67 index was higher in chondrosarcoma grade 3, MCS and DCS than chondrosarcoma grade 1/2. The comparison of IHC markers further highlighted the overexpression of P53/MDM2 in MCS and DCS. Gross total resection, including en-bloc and piecemeal resection, significantly improved OS and RFS for CCS patients, while only en-bloc resection significantly improved the prognosis of MCS patients. Chemotherapy appeared to be important for the OS of MCS patients. Conclusion P53/MDM2 pathway was upregulated in MCS and DCS compared to chondrosarcoma grade 1/2. Radical tumor resection is crucial for the treatment of spinal chondrosarcoma, while MCS patients require further comprehensive treatments perioperatively.

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.

Chondrosarcoma is the third most common primary malignant bone tumour. There are various histological subtypes of chondrosarcomas, of which conventional intramedullary chondrosarcoma is by far the most common. Rarer sub-types include clear cell chondrosarcoma, myxoid chondrosarcoma, mesenchymal chondrosarcoma and dedifferentiated chondrosarcoma. Chondrosarcoma is also classified into central, peripheral and periosteal, dependent upon the lesion site, and into primary chondrosarcoma if the lesion arises de novo and secondary chondrosarcoma if the tumour arises in a pre-existing lesion. The various subtypes of chondrosarcoma have characteristic imaging features that may aid diagnosis and may guide biopsy, therefore potentially preventing misdiagnosis. The aim of this article is to provide an overview of the pertinent clinical and imaging findings of the different forms of chondrosarcoma.

Background Changes in gut microbiota metabolism might play an important role in the development of some cancers. However, the causal relationships of gut microbiome-related metabolic pathways in chondrosarcomas and the specific pathways affected remain largely unknown. Methods We used two-sample bidirectional and multivariate Mendelian randomization (MR) to reveal a causal relationship between the gut microbiota metabolic pathway (GMMP) and chondrosarcoma associated gene 1(CSAG1) via the largest available genome-wide association study (GWAS). Results Univariate MR analysis revealed that tetrapyrrole biosynthesis from glutamate, menaquinol 6 biosynthesis, glycogen degradation II, 8-amino-7-oxononanoate biosynthesis, taxadiene biosynthesis, glycolysis and tRNA charging had a significant causal relationship with CSAG1.Multivariate MR analysis suggested that tetrapyrrole biosynthesis, menaquinol 6 biosynthesis, glycogen degradation II, glycolysis and tRNA charging still had a significant causal effect on CSAG1. According to the results of reverse MR analysis, no significant causal effect of CSAG1 on the GMMP was found. Conclusions This study offers further insights into the gut microbiota-mediated mechanism of chondrosarcoma development.

Chondrosarcomas are rare cartilaginous neoplasms with limited treatment options. Isocitrate dehydrogenase 1/2 (m IDH1/2 ) mutations occur in 65% of chondrosarcomas. Here we report safety and efficacy of olutasidenib, an mIDH1 inhibitor, evaluated in patients with locally advanced or metastatic m IDH1 chondrosarcoma (Clinicaltrials.gov identifier: NCT03684811). The primary endpoint was objective response rate by tumor evaluation; secondary endpoints included adverse events, progression-free and overall survival. Patients received olutasidenib 150 mg twice daily. Twenty-three patients were enrolled; 16 were diagnosed with conventional chondrosarcoma (cCS). Median age was 57 (range, 30-71) years. In 21 response-evaluable patients, 11 (52%) had stable disease, 8 (38%) had progressive disease, and 2 (10%) were not evaluable. Median progression-free survival (mPFS) was 2.0 months (95% confidence interval [95%CI]: 1.7, 4.7); median overall survival was 16.0 months (95%CI: 7.7, not reached). Among patients with cCS, 10 (63%) had stable disease; 6 (38%) had progressive disease; mPFS was 3.5 months (95%CI: 1.7, 5.1). Median overall survival in cCS patients was 19.0 months (95% CI: 7.7, not reached). No dose-limiting toxicities were reported during the study. Olutasidenib was well tolerated and conferred disease control in cCS. Study limitations include open-label design and low patient sample due to rarity of cCS. Chondrosarcomas are rare cartilaginous neoplasms with limited treatment options. Here this trial evaluates the safety and efficacy of olutasidenib (mIDH1 inhibitor) on 23 patients with locally advanced or metastatic mIDH1 chondrosarcoma.

Introduction Percutaneous cryoablation is a first-line therapeutic option for primary neoplasms and metastatic lesions of the musculoskeletal system. Treatment of abdominal wall tumors is challenging as surgical resection can be highly morbid and necessitate complex reconstructive surgery; the efficacy of cryoablation for abdominal wall tumors may be limited by inadequate posterior margins owing to the proximity of intra-abdominal organs. With laparoscopy and insufflation, peritoneal structures can be safely mobilized away from the abdominal wall, allowing for adequate deep margin freeze and visualization of the ice ball. We present two patients with abdominal wall soft tissue tumors treated with a novel approach of laparoscopic-assisted, percutaneous ultrasound-guided cryoablation. Patients and Methods Patient 1 is a 65-year-old female with metastatic extraskeletal myxoid chondrosarcoma, stable on systemic therapy, who presented with a new soft tissue metastasis to the abdominal wall. Resection would have necessitated a highly morbid complex abdominal wall reconstruction with mesh. Patient 2 is a 35-year-old female with a large abdominal wall desmoid tumor, diagnosed after miscarriage. Resection was relatively contraindicated owing to the morbidity of a complex abdominal wall reconstruction and concerns regarding potential future pregnancies after surgery. Results Both patients underwent procedures in the outpatient setting after discussion at multidisciplinary sarcoma tumor board. Laparoscopic enterolysis was performed to mobilize the bowel away from the abdominal wall, to allow direct visualization of the peritoneal aspect of the tumor, and to confirm adequacy of the posterior margin freeze of the lesion. Laparoscopic transversus abdominus preperitoneal (TAPP) blocks with local anesthetic were performed for postoperative pain control. Interventional radiology performed an ultrasound-guided cryoablation consisting of two freeze and thaw cycles. Both patients recovered well without complications and were without radiographic evidence of persistent or recurrent disease at 12 and 18 months postoperatively, respectively. Conclusion We report a novel approach of laparoscopic-assisted cryoablation for the treatment of abdominal wall soft tissue tumors. This allowed for successful minimally invasive local control of these large tumors that would have otherwise required highly morbid resections with complex abdominal wall reconstruction and mesh repair.