Chordoma

01/01/2023 - "The purpose of this study is to report the mid-term results of primary spinal chordoma treated with en bloc resection and proton-based RT with minimum 5 years of follow-up. "
01/01/2023 - "Pediatric patients with BOS chordomas were treated with PRT or combined proton/photon approach (proton-based; for most, 80% proton/20% photon) at the Massachusetts General Hospital from 1981 to 2021. "
01/01/2023 - "We present efficacy and toxicity outcomes among patients with chordoma treated on the Proton Collaborative Group prospective registry. "
12/01/2022 - "Proton beam radiation is increasingly being used as a postoperative radiation modality for the treatment of chordomas."
10/18/2022 - "The management of clival chordoma in our group shifted around 2013 to mostly endoscopic, and proton beam was introduced for our multidisciplinary team. "

01/01/2020 - "Fast Clearance of the SARS-CoV-2 Virus in a Patient Undergoing Vaccine Immunotherapy for Metastatic Chordoma: A Case Report."
01/01/2021 - "Defects in HLA Class I expression may play a role in the clinical course of chordoma and may account for the limited or lack of efficacy of T cell-based immunity triggered by vaccines and/or checkpoint inhibitors."
12/30/2022 - "The brachyury vaccine has shown good safety in chordoma patients, and future clinical trials should focus on how to improve its therapeutic efficacy. "
06/07/2016 - "Clinical trials are ongoing in which chordoma patients are treated with brachyury-specific vaccines. "
01/01/2021 - "Randomized, Double-Blind, Placebo-Controlled Phase II Study of Yeast-Brachyury Vaccine (GI-6301) in Combination with Standard-of-Care Radiotherapy in Locally Advanced, Unresectable Chordoma."

05/18/2023 - "Our preclinical studies demonstrate the potential of VLP-based Cas9/gRNA RNP gene therapy for the treatment of brachyury-dependent chordoma."
11/01/2021 - "A limited number of studies that examined the mutational profiles in chordomas identified potential driver mutations, including duplication in the TBXT gene (encoding brachyury), mutations in the PI3K/AKT signaling pathway, and loss of the CDKN2A gene. "
08/01/2021 - "Thus, the aim of this study is to investigate the expression of brachyury expression in a series of chordomas in conjunction to clinicopathological characteristics and to review the relevant literature providing all the details needed in the immunohistochemical study of brachyury. "
01/01/2021 - "Some studies have reported high specificity and sensitivity of brachyury expression in chordomas. "
12/01/2015 - "However, recent advances in the molecular biology studies have identified brachyury underlying the initiation and progression of chordoma cells. "

06/01/1997 - "The expression of CK8 and CK19, observed in all of the chordoma and notochord specimens, was thus considered to be maintained throughout the neoplastic transformation, although some aberrant CK expressions (CK7, CK18, and keratin-903) also occurred in the chordoma specimens examined in this study."
01/01/2019 - "The vacuolated morphology of chordoma cells is dependent on cytokeratin intermediate filaments."
05/01/2010 - "Comment on: 'Expression of c-MET, low-molecular-weight cytokeratin, matrix metalloproteinases-1 and -2 in spinal chordoma'. "
04/01/2009 - "Expression of c-MET, low-molecular-weight cytokeratin, matrix metalloproteinases-1 and -2 in spinal chordoma."
11/01/2001 - "Cytokeratin immunophenotyping of an unusual cervical vertebral chordoma with extensive chondroid foci and perilaryngeal recurrence: a case report with review of the literature."

11/01/2014 - "Highly effective treatment of skull base chordoma with carbon ion irradiation using a raster scan technique in 155 patients: first long-term results."
08/24/2020 - "Our analysis showed only moderate long-term LC rates after carbon ion-based RT, with sacral chordomas having a particularly poor prognosis in the recurrent situation. "
08/23/2018 - "Sacral fractures after high-dose carbon ion-based RT of sacral chordomas were shown to be a considerable radiogenic late effect, affecting about half of the treated patients. "
07/01/2015 - "Carbon ion beam treatment in patients with primary and recurrent sacrococcygeal chordoma."
10/01/2014 - "Dosimetric effects of residual uncertainties in carbon ion treatment of head chordoma."

12/01/2008 - "Positivity for both epithelial membrane antigen and AE1/AE3 had a sensitivity of 90% and a specificity of 100% for detecting chordoma in this study. "
05/01/1998 - "Thus, positive staining with epithelial membrane antigen and the identification of a large number of rough endoplasmic reticulum-mitochondrial complexes are suggestive of metastatic chordoma. "
09/01/1987 - "Demonstration of cytokeratins and an epithelial membrane antigen in chondroid chordoma."
11/01/1985 - "Demonstration of cytokeratins and an epithelial membrane antigen in chordomas and human fetal notochord."
10/02/2018 - "Histology of the tumor was consistent with chordoma, supported by positive immunohistochemical staining for S-100 and epithelial membrane antigen (EMA). "

01/01/1994 - "Chondroid chordomas were stained for vimentin with moderate intensity whereas typical chordomas were only slightly stained. "
09/01/1991 - "The five chondroid chordomas exhibited the following pattern: EMA 0%, AE 1/3 0%, DPK 0%, vimentin 100%, S100 100%, NSE 100%, CEA 0%, and HMB-45 0%. "
01/01/1994 - "Vimentin and S-100 were positive in more than 95% of both classic and chondroid chordomas and chondrosarcomas. "
01/01/1993 - "In these cases, the expression of S-100-protein, vimentin and CEA by the chordoma cells and the typical electron microscopic features of chordomas are helpful for the differential diagnosis. "
01/01/2019 - "Immunohistochemistry revealed dual expression of cytokeratin AE1/AE3 and vimentin, consistent with a diagnosis of chordoma. "

11/15/1993 - "Forty-four percent of classic and 85% of chondroid chordomas were positive for S-100 protein. "
05/01/1988 - "Sixteen of the 20 chordomas (80%) stained for S100 protein, including all three chondroid chordomas. "
03/01/1983 - "Using immunohistochemical methods, we found S100 protein in human chordomas and in human and rabbit notochordal tissue. "
03/01/1983 - "S100 protein in human chordoma and human and rabbit notochord."
05/01/1997 - "Immunocytochemistry with positivity for S-100 protein and cytokeratins was an essential adjunct in the cytologic diagnosis of chordoma and helped in distinguishing it from other chondrogenic tumors. "

12/01/2016 - "Recent work published in the Journal of Pathology by Scheipl et al describing a focused compound drug screen highlights the significance of epidermal growth factor receptor (EGFR) signalling in chordoma, and shows potential for EGFR inhibitors as a way forward for developing an effective treatment for chordoma. "
10/04/2011 - "Efficacy of epidermal growth factor receptor targeting in advanced chordoma: case report and literature review."
01/01/2022 - "Optimization of the 4-anilinoquin(az)oline scaffold as epidermal growth factor receptor (EGFR) inhibitors for chordoma utilizing a toxicology profiling assay platform."
01/01/2019 - "Further investigation revealed that YBX1 enhanced epidermal growth factor receptor (EGFR) transcription by directly binding to its promoter in chordoma cells. "
01/01/2018 - "Expression of epidermal growth factor receptor (EGFR) in a large proportion of chordoma specimens indicates a potential target for therapeutic intervention. "

03/14/2022 - "Dramatic In Vivo Efficacy of the EZH2-Inhibitor Tazemetostat in PBRM1-Mutated Human Chordoma Xenograft."
03/14/2022 - "The first demonstration of a high antitumor activity of tazemetostat in a PDX model harboring a PBRM1 variant supports further evaluation for EZH2-inhibitors in this subgroup of chordomas."
01/01/2019 - "We sought to determine the mechanism of an exceptional response in a patient diagnosed with a SMARCB1/INI1-negative chordoma treated with tazemetostat, an EZH2 inhibitor, and followed by radiotherapy.Patient"
01/01/2018 - "In a phase I trial of the EZH2 inhibitor tazemetostat, children with INI1-deficient tumors-including relapsed or refractory malignant rhabdoid tumors, atypical teratoid rhabdoid tumors, epithelioid sarcomas, and poorly differentiated chordomas-responded well to treatment, with some experiencing durable responses."

06/01/2019 - "The long-term prognosis of sacral chordoma was the worst (10-year survival rate = 48%).The best treatment strategy for improved long-term survival in chordoma was a combination of surgical resection and radiation therapy. "
01/01/2018 - "For spinal chordomas, PBS-PT remains a highly effective and safe method for delivery of dose-escalated adjuvant radiation therapy. "
09/01/2015 - "The best treatment of sacral chordoma is surgical resection, nowadays associated with optimized radiation therapy. "
02/01/1999 - "The current 'best treatment method' for clival chordoma is regarded as radical surgical resection followed by radiation therapy; radiosurgery usually plays a major part in the radiation therapy programme. "
03/01/2015 - "Adjuvant high-dose stereotactic fractionated radiotherapy (HS-FSRT) significantly improved 5 year PFS in craniocervical chordoma patients (70%, n=13) as compared to standard dose radiation therapy (20%, n=16; p-value=0.03). "

01/01/2019 - "In DRT for chordoma, proton therapy was associated with improved OS at 5 years in a multivariate analysis (100% vs 34.1%; P = .031), and a high dose for chordoma was significant for improved OS in a univariate analysis with both DRT (79.0% vs 54.1%; P = .027) and PRT (83.3% vs 77.4%; P = .007). "
06/01/2014 - "Chordoma management is more widely studied with en bloc resection and adjuvant proton-beam therapy demonstrating improved progression-free survival and overall survival. "
12/04/2021 - "Therapeutic Efficacy of Variable Biological Effectiveness of Proton Therapy in U-CH2 and MUG-Chor1 Human Chordoma Cell Death."
12/01/2021 - "Our data indicate that pencil beam scanning proton therapy for sacral chordomas is both safe and effective. "
08/01/2016 - "To review the indications and efficacy of proton beam therapy in the management of skull base chordomas. "

01/01/1998 - "The disappointing results with photon therapy in a multimodality approach and the good results reported by Schoenthaler with charged particles in the Lawrence Berkeley laboratory led us to propose fast neutron therapy in the management of inoperable or recurrent sacrococcygeal chordomas. "
12/30/2022 - "No clinical data have demonstrated that CTLA-4 ICIs combined with PD-1/PD-L1 ICIs is more effective than ICIs monotherapy in treating chordoma, and ICIs in combination with other therapies exhibit more toxicity than monotherapy. "
09/01/2011 - "While no chemotherapeutic agent has been demonstrated to be effective against chordoma in vivo, a greater understanding of the genetics and molecular biology of chordoma is opening up avenues of investigation towards the rational development of targeted therapies. "
01/01/2023 - "Photon treatment is a safe and effective treatment for chordomas if no particle therapy is available. "
02/01/2022 - "Materials and Methods  An in vitro study on automated high-throughput screening of chordoma cells was performed using a library of 1,406 drugs as both mono- and combination therapies with PI3K inhibitors. "

05/01/2016 - "Carbon ion radiation therapy was safe and effective for unresectable chordoma and provided good local control and survival while preserving ambulation."
11/01/2014 - "Carbon ion therapy appears to be a safe and effective treatment for patients with skull base chordoma, resulting in high LC and OS rates."
12/01/2009 - "A carbon ion radiotherapy is an effective treatment for chordomas of the skull base with minimal side effects. "
05/01/2019 - "However, carbon ion radiotherapy (CIRT) has recently emerged as a promising treatment for unresectable sacral chordoma. "
08/01/2010 - "Carbon ion radiotherapy appears effective and safe in the treatment of patients with sacral chordoma and offers a promising alternative to surgery."

01/01/2022 - "Chordomas are considered insensitive to conventional chemotherapy, and their rarity complicates running timely and adequately powered trials to identify effective treatments. "
01/01/2018 - "Despite extensive studies, the mechanisms of chordoma recurrence after surgical intervention, as well as resistance to radiation and chemotherapy, remain elusive. "
08/01/1993 - "A trial of reasonably aggressive chemotherapy is warranted in patients with metastatic dedifferentiated chordoma. "
07/01/2023 - "Despite encouraging advances in radiation and surgical treatment, chordomas remain resistant to chemotherapy and local recurrence is common. "
04/01/2023 - "Furthermore, no alteration in the sensitivity of chordoma cells to chemotherapy was detected in response to the decrease in eEF2K expression which intriguingly promoted suppression of cell migratory and invasion related properties. "