Left Hepatectomy Enlarged to Segment 1 with Total Vascular Exclusion of the Liver Preserving the Caval Flow with Temporary Portacaval Shunt and Hypothermic Oxygenated Portal Perfusion on Machine for Metastatic Recurrence of a Pleural Chondrosarcoma. Total vascular exclusion (TVE) of the liver preserving the caval flow with portal hypothermic perfusion and temporary portacaval shunt (PCS metastatic pleural chondrosarcoma, with a pleural metastasis and several intra-abdominal metastases, including a lesion localized in segment 1 close to the hilar plate and invading the left HV near its abutment in the IVC. A left hepatectomy enlarged to segment 1 was performed under TVE preserving the caval flow with in situ hypothermic portal perfusion of the liver using perfusion machine. PV clamping
Phase I study of the mutant IDH1 inhibitor ivosidenib: long-term safety and clinical activity in patients with conventional chondrosarcoma. A phase I study demonstrated that ivosidenib, a mutant isocitrate dehydrogenase 1 (mIDH1) inhibitor, showed manageable toxicity and durable disease control in patients with mIDH1 conventional chondrosarcoma (CS). Here we present long-term follow-up data
Contemporary Surgical Approaches in Managing Laryngeal Chondrosarcoma: A Scoping Review. The aim of this scoping review is to describe and evaluate current surgical management options for chondrosarcoma of the larynx, and to summarize these techniques with respect to the laryngeal subsite affected. PubMed/MEDLINE and EMBASE databases. The review was conducted according to the Preferred Reporting Items for Systematic Reviews extension for Scoping Reviews. Forty-one articles met eligibility criteria for inclusion in the final analysis, which included a total of 149 unique surgical cases that were analyzed based on affected laryngeal subsites of cricoid, thyroid, arytenoid, and epiglottis. Management of cricoid chondrosarcoma favored transoral endoscopic resection (TER) or transcervical
Identifying consensus and areas for future research in chondrosarcoma : a report from the Birmingham Orthopaedic Oncology Meeting. The Birmingham Orthopaedic Oncology Meeting (BOOM), held in January 2024, convened 309 delegates from 53 countries to discuss and refine 21 consensus statements on the optimal management of chondrosarcoma. With representation from Europe (43%; n = 133), North America statements, reflecting agreement among delegates. Key areas of consensus included the role of radiology in diagnosis and surveillance, the management of locally recurrent disease, and the treatment of dedifferentiated chondrosarcoma. Notably, there was agreement that routine chemotherapy has no role in chondrosarcoma treatment, and radiological surveillance is safe for intraosseous chondrosarcomas. Despite
Long-term Outcomes following Proton-based Radiation Therapy for Pediatric Chordomas and Chondrosarcomas of the Mobile Spine and the Sacrum. To report the first cohort of children with spinal and sacrococcygeal chordomas (CH) and chondrosarcomas (CHS) treated with proton-based radiotherapy (PRT). Between 1989 and 2019, 52 pediatric patients ≤ 22 years old with spinal CH (n=43) or CHS (n=9) were
Complications and local recurrence of chondrosarcoma and chordoma treated by total tumor resection in thoracic and lumbar spine. En bloc resection of spinal tumors is challenging and associated with a high incidence of complications; however, it offers the potential to reduce the risk of recurrence when a wide margin is achieved. This research aims to investigate the safety and efficacy of en bloc resection in treating thoracic and lumbar chondrosarcoma/chordoma. Data from patients diagnosed with chondrosarcoma and chordoma in the thoracic or lumbar region, who underwent total en bloc or piecemeal resection at our institution over a 7-year period, were collected and regularly followed up. The study analyzed overall perioperative complications and compared differences in complications
A personalized medicine approach identifies enasidenib as an efficient treatment for IDH2 mutant chondrosarcoma. Sarcomas represent an extensive group of malignant diseases affecting mesodermal tissues. Among sarcomas, the clinical management of chondrosarcomas remains a complex challenge, as high-grade tumours do not respond to current therapies. Mutations in the isocitrate dehydrogenase (IDH ) 1 and 2 genes are among the most common mutations detected in chondrosarcomas and may represent a therapeutic opportunity. The presence of mutated IDH (mIDH) enzymes results in the accumulation of the oncometabolite 2-HG leading to molecular alterations that contribute to drive tumour growth. We developed a personalized medicine strategy based on the targeted NGS/Sanger sequencing of sarcoma
Are IDH1 R132 Mutations Associated With Poor Prognosis in Patients With Chondrosarcoma of the Bone? Because chondrosarcomas vary widely in their behavior, and because anticipating their behavior based on histology alone can be challenging, genetic markers represent an appealing area of inquiry that may help us refine our prognostic approaches. Isocitrate dehydrogenase (IDH) mutations are involved in the pathogenesis of a variety of neoplasms, and recently, IDH1/2 mutations have been found in the tissue of benign cartilage tumors as well as in conventional chondrosarcomas and highly aggressive dedifferentiated chondrosarcomas. However, their association with patient survival is still controversial. (1) What proportion of patients with chondrosarcomas carry IDH mutations, and which IDH
X-rays radiomics-based machine learning classification of atypical cartilaginous tumour and high-grade chondrosarcoma of long bones. Atypical cartilaginous tumour (ACT) and high-grade chondrosarcoma (CS) of long bones are respectively managed with active surveillance or curettage and wide resection. Our aim was to determine diagnostic performance of X-rays radiomics-based machine learning
Chondrosarcoma of the Mobile Spine: An Update on Patients Treated at a Single Institution. Retrospective study. The objective is to report the clinical data for patients treated with mobile spine chondrosarcoma. Chondrosarcoma of the mobile spine is a rare and challenging entity. A handful of case series have been published that report the clinical results of treatment, largely influenced by chondrosarcoma of the appendicular skeleton and pelvis. The clinical results of patients treated for chondrosarcoma of the mobile spine from our institution were published over ten years ago and this represents and update since that publication. Inclusion criteria were adults patients treated for chondrosarcoma of the mobile spine at Massachusetts General Hospital between 2007-2020. Patients with large sacral
Proton-Based Radiation Therapy for Skull Base Chondrosarcomas in Children and Adolescents: 40-year experience from a single institution. The literature on skull base chondrosarcoma (CHS) is scarce. We report outcomes for pediatric and young patients ≤ 22 years with base of skull (BOS) CHSs treated with proton-based radiotherapy (PBRT). We retrospectively reviewed all patients treated with PBRT between 1981 and 2023. Primary endpoints were overall survival (OS), chondrosarcoma-specific survival (CSS), progression-free survival (PFS), local control (LC), and distal control (DC). Eighty-four patients were identified. Median age at diagnosis was 19 years (range, 6 - 22). Most patients (n=79, 94%) had conventional CHS while 5 (6%) had mesenchymal CHS. Nine patients (11%) underwent gross total
Enhancing surgical margins: A key to survival in pelvic chondrosarcoma. Pelvic chondrosarcoma, originating in the cartilaginous tissue of the pelvis, presents unique surgical challenges due to its proximity to critical neurovascular structures and essential organs. The aim of this study was to explore the effect of changes in practice and improvements in understanding on the outcomes for patients undergoing surgery for pelvic chondrosarcoma. The study comprised a retrospective analysis of 216 patients making up 2 patient cohorts drawn from a single centre over 2 time periods (2003-2012 and 2013-2022). The results demonstrate significant improvements in disease-specific survival (DSS) and local recurrence-free survival (LRFS) in the modern cohort. Contributors to this improvement include
Chondrosarcoma of the Flat Bones: Differential Survival Between High-Grade Lesions of the Pelvis and Scapula. Flat bone chondrosarcomas have worse outcomes than extremity tumors, but there is no data directly comparing the different flat bones. The aim of this study was to examine differences in recurrence and survival between pelvic and scapular chondrosarcoma. One hundred and sixty-nine (42 scapula, 127 pelvic) patients with chondrosarcoma who underwent surgical resection were reviewed. High-grade tumors were defined as lesions that were Grade 3 or dedifferentiated on surgical pathology. Patients with low-intermediate grade lesions of the scapula were more likely to have positive margins during definitive surgical management (14% vs. 3%, OR 5, 95% CI [1.15, 22.6], p = 0.02), however
The Impact of Chondrosarcoma with Positive Margins and Extraosseous Extension on Patient Outcomes. Chondrosarcoma accounts for 20% of all bony sarcomas and may present with extraosseous extension (EOE). The presence of an extraosseous component, along with positive surgical margins, has been separately associated with increased risk of local recurrence and decreased survival. This study compared the outcomes between patients with EOE, EOE and positive margins, and a control chondrosarcoma cohort with neither feature. This was a retrospective review of 91 patients over a consecutive 13-year period. Data including treatment details and outcomes were included. Thirty-two patients had EOE of their chondrosarcoma, 7 patients had positive margins and EOE, and 52 chondrosarcoma patients had neither
Complete excision of a giant chondrosarcoma within the cavernous sinus: a case report and literature review. Primary skull base chondrosarcoma (SBC) is a rare malignant central nervous system tumor, often involving the cavernous sinus. Complete excision of tumors invading this region is exceptionally challenging due to the presence of the internal carotid artery and numerous nerves within carotid artery was observed without new severe complications. The postoperative pathological diagnosis, according to the World Health Organization classification, was Grade 1 chondrosarcoma; therefore, radiotherapy was not administered. Magnetic resonance imaging at the 8-month follow-up showed no residual tumor or recurrence. This case highlights that surgical complete excision of large intracavernous
The diagnostic value of magnetic resonance imaging-based texture analysis in differentiating enchondroma and chondrosarcoma. To assess the diagnostic performance of MRI-based texture analysis for differentiating enchondromas and chondrosarcomas, especially on fat-suppressed proton density (FS-PD) images. The whole tumor volumes of 23 chondrosarcomas and 24 enchondromas were manually segmented for T1-weighted, FS-PD, and T1-weighted + FS-PD images were selected in terms of accuracy and area under the curve (AUC). There were 7 men and 16 women in the chondrosarcoma group (mean ± standard deviation age, 45.65 ± 11.24) and 7 men and 17 women in the enchondroma group (mean ± standard deviation age, 46.17 ± 11.79). Naive Bayes was the best model for accuracy and AUC for T1-weighted images (AUC
Is a radiological score able to predict resection-grade chondrosarcoma in primary intraosseous lesions of the long bones? The preoperative grading of chondrosarcomas of bone that accurately predicts surgical management is difficult for surgeons, radiologists, and pathologists. There are often discrepancies in grade between the initial biopsy and the final histology. Recent advances in the use of imaging methods have shown promise in the ability to predict the final grade. The most important clinical distinction is between grade 1 chondrosarcomas, which are amenable to curettage, and resection-grade chondrosarcomas (grade 2 and 3) which require en bloc resection. The aim of this study was to evaluate the use of a Radiological Aggressiveness Score (RAS) to predict the grade of primary
Construction of novel predictive tools for post-surgical cancer-specific survival probability in patients with primary chondrosarcoma and external validation in Chinese cohorts: a large population-based retrospective study. Surgery is the predominant treatment modality for chondrosarcoma. This study aims to construct a novel clinic predictive tool that accurately predicts the 3-, 5-, and 8-year probability of cancer-specific survival (CSS) for primary chondrosarcoma patients who have undergone surgical treatment. The Surveillance, Epidemiology, and End Results (SEER) database was used to identify 982 primary chondrosarcoma patients after surgery, who were randomly divided into two sets: training set (60%) and internal validation set (40%). Cox proportional regression analyses were used to screen
Melatonin inhibits chondrosarcoma cell proliferation and metastasis by enhancing miR-520f-3p production and suppressing MMP7 expression. Chondrosarcoma has a high propensity to metastasize and responds poorly to chemotherapy and radiation treatment. The enzymatic activity of matrix metalloproteinases (MMPs) is very important in chondrosarcoma metastasis. Melatonin exhibits anticarcinogenic activity in many types of cancers by suppressing the expression of certain MMP family members, but this has not yet been clearly determined in chondrosarcoma. Our study demonstrates that MMP7 plays an essential role in chondrosarcoma cell proliferation, migration and anoikis resistance. We also found that MMP7 is highly expressed in chondrosarcomas. Our in vitro and in vivo investigations show
The national incidence of chondrosarcoma of bone; a review. Chondrosarcoma (CS) epidemiology has been studied by a number of authors using national cancer registry cohorts. Many reports share the common findings of a slight increase in incidence, but not all. The patterns and causes for these changes are divergent while reflection concerning methodological challenges are often missing. We have