Sacral Chordoma: A Population-based Analysis of Epidemiology and Survival Outcomes

Scampa, Matteo; Tessitore, Enrico; Dominguez, Dennis; Hannouche, Didier; Buchs, Nicolas; Kalbermatten, Daniel F.; Oranges, Carlo M.

doi:10.21873/anticanres.15552

Cited by 9 publications

(11 citation statements)

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“…However, chordomas do occur in the mobile spine as well. Although pathologically benign in appearance and generally slow growing with a median overall survival of approximately a decade [ 1 ], these tumors are considered malignant as they have metastatic potential. Specifically, 5–40% of patients develop distant metastases during their disease course [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Radiotherapy for Mobile Spine and Sacral Chordoma: A Critical Review and Practical Guide from the Spine Tumor Academy

Redmond

Schaub

et al. 2023

Cancers

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Chordomas are rare tumors of the embryologic spinal cord remnant. They are locally aggressive and typically managed with surgery and either adjuvant or neoadjuvant radiation therapy. However, there is great variability in practice patterns including radiation type and fractionation regimen, and limited high-level data to drive decision making. The purpose of this manuscript was to summarize the current literature specific to radiotherapy in the management of spine and sacral chordoma and to provide practice recommendations on behalf of the Spine Tumor Academy. A systematic review of the literature was performed using the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) approach. Medline and Embase databases were utilized. The primary outcome measure was the rate of local control. A detailed review and interpretation of eligible studies is provided in the manuscript tables and text. Recommendations were defined as follows: (1) consensus: approved by >75% of experts; (2) predominant: approved by >50% of experts; (3) controversial: not approved by a majority of experts. Expert consensus supports dose escalation as critical in optimizing local control following radiation therapy for chordoma. In addition, comprehensive target volumes including sites of potential microscopic involvement improve local control compared with focal targets. Level I and high-quality multi-institutional data comparing treatment modalities, sequencing of radiation and surgery, and dose/fractionation schedules are needed to optimize patient outcomes in this locally aggressive malignancy.

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Section: Introductionmentioning

confidence: 99%

Radiotherapy for Mobile Spine and Sacral Chordoma: A Critical Review and Practical Guide from the Spine Tumor Academy

Redmond

Schaub

et al. 2023

Cancers

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“…Successful treatment of chordomas requires an experienced multidisciplinary team. The median overall survival of patients with chordoma is between 6.3 and 7.7 years ( 1 , 2 ) with a 5-year overall survival around 70% in most series ( 2 – 4 ). For newly diagnosed patients, primary treatment consists of radical but safe resection; importantly, the extent of resection directly impacts outcomes in patients with skull base and spine chordomas.…”

Section: Introductionmentioning

confidence: 99%

Prognostic molecular biomarkers in chordomas: A systematic review and identification of clinically usable biomarker panels

Rubino

Alvarez‐Breckenridge²,

Akdemir³

et al. 2022

Front. Oncol.

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Introduction and objectiveDespite the improvements in management and treatment of chordomas over time, the risk of disease recurrence remains high. Consequently, there is a push to develop effective systemic therapeutics for newly diagnosed and recurrent disease. In order to tailor treatment for individual chordoma patients and develop effective surveillance strategies, suitable clinical biomarkers need to be identified. The objective of this study was to systematically review all prognostic biomarkers for chordomas reported to date in order to classify them according to localization, study design and statistical analysis.MethodsUsing the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, we systematically reviewed published studies reporting biomarkers that correlated with clinical outcomes. We included time-to-event studies that evaluated biomarkers in skull base or spine chordomas. To be included in our review, the study must have analyzed the outcomes with univariate and/or multivariate methods (log-rank test or a Cox-regression model).ResultsWe included 68 studies, of which only 5 were prospective studies. Overall, 103 biomarkers were analyzed in 3183 patients. According to FDA classification, 85 were molecular biomarkers (82.5%) mainly located in nucleus and cytoplasm (48% and 27%, respectively). Thirty-four studies analyzed biomarkers with Cox-regression model. Within these studies, 32 biomarkers (31%) and 22 biomarkers (21%) were independent prognostic factors for PFS and OS, respectively.ConclusionOur analysis identified a list of 13 biomarkers correlating with tumor control rates and survival. The future point will be gathering all these results to guide the clinical validation for a chordoma biomarker panel. Our identified biomarkers have strengths and weaknesses according to FDA’s guidelines, some are affordable, have a low-invasive collection method and can be easily measured in any health care setting (RDW and D-dimer), but others molecular biomarkers need specialized assay techniques (microRNAs, PD-1 pathway markers, CDKs and somatic chromosome deletions were more chordoma-specific). A focused list of biomarkers that correlate with local recurrence, metastatic spread and survival might be a cornerstone to determine the need of adjuvant therapies.

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“…In practice, when PS is finally diagnosed, it usually has a large tumor volume due to the large and deep pelvic space and the lack of anatomic barriers to tumor diffusion, which often results in compression of adjacent organs, blood vessels, and nerves, making local control difficult 15,16 . Previous studies have been performed on the survival rates and the related prognostic factors of pelvic osteosarcoma, chondrosarcoma, Ewing sarcoma, and chordoma, but not on PS as a whole 17–20 . Although these individual studies provide helpful information for patients with specific PSs, the individual results of these specific tumors limit their application to medical decision‐makers in the overall management of PSs.…”

Section: Introductionmentioning

confidence: 99%

“… 15 , 16 Previous studies have been performed on the survival rates and the related prognostic factors of pelvic osteosarcoma, chondrosarcoma, Ewing sarcoma, and chordoma, but not on PS as a whole. 17 , 18 , 19 , 20 Although these individual studies provide helpful information for patients with specific PSs, the individual results of these specific tumors limit their application to medical decision‐makers in the overall management of PSs. Therefore, a study of patients with primary PS is required to identify the variables most closely associated with survival to assist clinicians in improving patient counseling and achieving risk‐stratified management and personalized care.…”

Section: Introductionmentioning

confidence: 99%

A novel clinical tool to predict cancer‐specific survival in patients with primary pelvic sarcomas: A large population‐based retrospective cohort study

Huang

Ding

et al. 2022

Cancer Medicine

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Background: Primary osseous sarcoma of the pelvis is rare and has a particularly sinister outcome. This study aims to identify independent prognostic factors of cancer-specific survival (CSS) in patients with primary pelvic sarcoma (PS) and develop a nomogram to predict 3-, 5-, and 10-year probability of CSS in these patients. Methods:The Surveillance, Epidemiology, and End Results (SEER) database was used to identify 416 patients with primary PS, who were divided into two groups: a training cohort and a validation cohort. Univariate and multivariate Cox analyses were used to screen independent prognostic factors in patients with primary PS. Based on these independent prognostic factors, a prognostic nomogram was developed to predict 3-, 5-, and 10-year probability of CSS. The nomogram's predictive performance and clinical value were evaluated using the calibration curve, receiver operating characteristic (ROC) curve, and decision curve analysis (DCA). Finally, a mortality risk stratification system was developed.Results: Tumor size, tumor stage, histological type, surgery, and chemotherapy were identified as independent prognostic factors for the CSS of primary PS patients. Based on these factors, a nomogram was created to predict the 3-, 5-, and 10-year probability of CSS in these patients. The calibration curve, ROC, and DCA indicated that the nomogram performed well and was appropriate for clinical use, with 3-, 5-, and 10-year areas under ROC curve all higher than 0.800. Furthermore, the nomogram-based mortality risk stratification system could effectively divide these patients into three risk subgroups. Conclusions:The nomogram constructed in this study could accurately predict 3-, 5-, and 10-year probability of CSS in patients with primary PS. Clinicians can use the nomogram to categorize these patients into risk subgroups and provide personalized treatment plans.

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Sacral Chordoma: A Population-based Analysis of Epidemiology and Survival Outcomes

Cited by 9 publications

References 0 publications

Radiotherapy for Mobile Spine and Sacral Chordoma: A Critical Review and Practical Guide from the Spine Tumor Academy

Radiotherapy for Mobile Spine and Sacral Chordoma: A Critical Review and Practical Guide from the Spine Tumor Academy

Prognostic molecular biomarkers in chordomas: A systematic review and identification of clinically usable biomarker panels

A novel clinical tool to predict cancer‐specific survival in patients with primary pelvic sarcomas: A large population‐based retrospective cohort study

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