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Background Chordoma is a rare and aggressive primary bone sarcoma. En-block resection remains the primary treatment, but some patients are unable to undergo it due to the location and potential complications. Currently, there is no direct comparison of the effects of radiotherapy (RTH) and surgical treatment. However, retrospective analyses indicate the potential benefits of using RTH. Methods A retrospective analysis was conducted on 48 patients with sacral chordoma who were treated with surgery and/or radiotherapy between 2001-2020. Among those, 22 were initially treated with surgery, 19 with definitive radiotherapy, and 7 received combined treatment. The outcomes of the treatment of recurrence in 16 patients were considered. The resection margins were defined according to R classification, and the Kaplan-Meier method was employed to calculate disease-free survival (DFS) and overall survival (OS). Results The median (mOS) for the entire cohort was 80.6 months (95% CI: 62.3-NA), and the median (mDFS) was 40.4 months (95% CI: 35-69.5). Patients who underwent radical surgery of the primary tumor did not achieve the mOS (mean 68), while patients treated only with RTH for the primary tumor achieved an mOS of 62.3 months (95% CI: 52.1-NA). This resulted in a significant advantage of surgery over RTH in terms of OS (P = 0.01). This was not observed for DFS. The 3-year DFS rates were 65% in the surgical treatment group and 53.3% in the RTH group. The 3-year OS rates were 96% in the surgery group and 88.9% in the RTH group. In the treatment of recurrence, there were no statistically significant differences between RTH and surgery, for OS (P = 0.76). Conclusions Radical surgery remains the optimal treatment for sacral chordoma. For patients who are not candidates for surgical intervention, RTH offers excellent long-term outcomes. The treatment of recurrence remains a significant challenge. Comparing modern RTH techniques and surgical procedures could provide further insights.

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.

Selection of cancer patients for treatment with immune checkpoint inhibitors remains a challenge due to tumour heterogeneity and variable biomarker detection. PD-L1 expression in 24 surgical chordoma specimen was determined immunohistochemically with antibodies 28-8 and E1L3N. The ability of patient-derived organoids to detect treatment effects of nivolumab was explored by quantitative and qualitative immunofluorescence and FACS analysis. The more sensitive antibody, E1L3N (ROC = 0.896, p  = 0.001), was associated with greater tumour diameters ( p  = 0.014) and detected both tumour cells and infiltrating lymphocytes in 54% of patients, but only 1–15% of their cells. Organoids generated from PD-L1-positive patients contained both tumour cells and PD-1/CD8-positive lymphocytes and responded to nivolumab treatment with marked dose-dependent diameter reductions of up to 50% and increased cell death in both PD-L1-positive and negative organoids. Patient-derived organoids may be valuable to predict individual responses to immunotherapy even in patients with low or no immunohistochemical PD-L1 expression.

Purpose The aim of this retrospective study is to analyze the impact of en bloc resection with negative margins versus intralesional resection plus adjuvant hadron-therapy (HT) on local control (LC) and overall survival (OS) in patients with mobile spine chordomas. Mechanical complications incidence as well as risk factors, and outcome differences are investigated as secondary endpoints. Methods 33 patients in a period from January 2013 to December 2021 were enrolled for the final analysis. The inclusion criteria were: lesions located in the mobile spine (C1-L5), age ≥ 15 years, minimum follow-up of 2 years, en bloc or intralesional surgical resection, virgin or recurrent chordomas, with only one previous surgical treatment. Results No difference was found in terms of LC between the two groups. The presence of pathologic fracture at pre-operative imaging and the presence of macroscopic residual tumor after surgery, independently from its entity, seemed to be associated with an increased risk of LR. No difference was found between planned en bloc and planned intralesional surgery in terms of mechanical complications occurrence. Eight patients (24.24%) had mechanical complications during the follow up period: male sex, presence of pathologic fracture at baseline, a combined surgical approach, the use of carbon fiber-only hardware appeared to be associated with an increased risk of mechanical complications after the primary surgery. Conclusions En bloc resection, whenever possible, is always to be preferred for its widely recognized potential in LC and OS improvement. However, technology advances in high-dose conformal charged-particle therapy have allowed improvement of local control rates as an adjuvant therapy of intralesional surgery for mobile spine chordoma, with acceptable acute and chronic toxicity.

Introduction Despite their precarious behavioral classification (benign and low grade on histopathology yet behaviorally malignant), great strides have been taken to improve prognostication and treatment paradigms for patients with skull base chordoma. With respect to surgical techniques, lateral transcranial (TC) approaches have traditionally been used, however endoscopic endonasal approaches (EEA) have been advocated for midline lesions. Nonetheless, due to the rarity of this pathology (0.2% of all intracranial neoplasms), investigations within the literature remain limited to small retrospective series. Furthermore, radiotherapeutic treatments investigated to date have proven largely ineffective. Methods Accordingly, we performed a systematic review in order to profile surgical and survival outcomes for skull base chordoma. Fixed and random-effect meta-analyses were performed for categorical variables including GTR, STR, 5-year OS, 10-year OS, 5-year PFS, and 10-year PFS. Additionally, we pooled eligible studies for formal meta-analysis to compare outcomes by surgical approach (lateral versus midline). Statistical analyses were performed using R Studio ‘metafor’ package or Cochrane Review Manager. Furthermore, meta-analysis of pooled mortality rates and sub-analyses of operative margin and surgical complications were used to compare midline versus lateral approaches via the Mantel-Haenszel method. We considered all p-values < 0.05 to be statistically significant. Results Following the systematic search and screen, 55 studies published between 1993 and 2022 reporting data for 2453 patients remained eligible for analysis. Sex distribution was comparable between males and females, with a slight predominance of male-identifying patients (0.5625 [95% CI: 0.5418; 0.3909]). Average age at diagnosis was 42.4 ± 12.5 years, while average age of treatment initiation was 43.0 ± 10.6 years. Overall, I 2 value indicated notable heterogeneity across the 55 studies [I 2  = 56.3% (95%CI: 44.0%; 65.9%)]. With respect to operative margins, the rate of GTR was 0.3323 [95% CI: 0.2824; 0.3909], I 2  = 91.9% [95% CI: 90.2%; 93.4%], while the rate of STR was significantly higher at 0.5167 [95% CI: 0.4596; 0.5808], I 2  = 93.1% [95% CI: 91.6%; 94.4%]. The most common complication was CSF leak (5.4%). In terms of survival outcomes, 5-year OS rate was 0.7113 [95% CI: 0.6685; 0.7568], I 2  = 91.9% [95% CI: 90.0%; 93.5%]. 10-year OS rate was 0.4957 [95% CI: 0.4230; 0.5809], I 2  = 92.3% [95% CI: 89.2%; 94.4%], which was comparable to the 5-year PFS rate of 0.5054 [95% CI: 0.4394; 0.5813], I 2  = 84.2% [95% CI: 77.6%; 88.8%] and 10-yr PFS rate of 0.4949 [95% CI: 0.4075; 0.6010], I 2  = 14.9% [95% CI: 0.0%; 87.0%]. There were 55 reported deaths for a perioperative mortality rate of 2.5%. The relative risk for mortality in the midline group versus the lateral approach group did not indicate any substantial difference in survival according to laterality of approach (-0.93 [95% CI: -1.03, -0.97], I 2  = 95%, (p < 0.001). Conclusion Overall, these results indicate good 5-year survival outcomes for patients with skull base chordoma; however, 10-year prognosis for skull base chordoma remains poor due to its radiotherapeutic resistance and high recurrence rate. Furthermore, mortality rates among patients undergoing midline versus lateral skull base approaches appear to be equivocal.

Purpose Chordomas are rare malignant neoplasms primarily treated surgically. Disparities related to race and socioeconomic status, may affect patient outcomes. This study aims to identify prognostic factors for access to care and survival in patients with spinal chordomas. Methods The NCDB database was queried between the years 2004 and 2017. Kaplan-Meier curves were constructed to compare survival probabilities among different groups, based on race and socioeconomic determinents. Results 1769 patients were identified, with 87% being White, 5% Hispanic, 4% Black, and Asian each. The mean age was 61.3 years. Most patients received care at academic/research centers and lived in a large metropolitan area, with no difference between races. A significantly higher percentage of Black patients did not undergo surgery ( p  < 0.001), with no statistically significant difference in survival between races ( p  = 0.97). A higher survival probability was seen in patients with other government insurances ( p  < 0.0001), in higher income quartiles ( p  < 0.0001), in metropolitan areas ( p  = 0.023), and at an academic/research center ( p  < 0.0001). A lower survival probability was seen in patients who are uninsured, in rural areas, and at community cancer programs ( p  < 0.0001). Conclusion This study highlights disparities in access to surgical intervention for patients with spinal chordomas, especially among Black individuals. It emphasizes the significant impact of insurance status and income on access to surgical care and highlights geographical and institutional variations in survival rates. Addressing socioeconomic differences is crucial for fostering equity in neurosurgical outcomes.

Purpose Sacral chordomas (SC) are rare, locally invasive, malignant neoplasms. Despite surgical resection and adjuvant therapies, local recurrence (LR) is common and overall survival (OS) is poor. The objective of this study was to identify prognostic factors that have an impact on the local recurrence-free survival (LRFS) and OS of patients with SC. Methods Utilizing the AOSpine Knowledge Forum Tumor multicenter ambispective database, surgically treated SC cases were identified. Cox regression modeling was used to assess the effect of several clinically relevant variables on OS and LRFS. Results A total of 167 patients with surgically treated SC were identified. The male/female ratio was 98/69 with a mean age of 57 ± 15 years at the time of surgery. The LR was 35 % ( n  = 57), death occurred in 30 % of patients ( n  = 50) during the study period. The median OS was 6 years post-surgery and LRFS was 4 years. In the univariate analysis, previous tumor surgery at the same site ( P  = 0.002), intralesional resection ( P  < 0.001), and larger tumor volume ( P  = 0.030) were significantly associated with LR. Increasing age ( P  < 0.001) and a preoperative motor deficit of C or D ( P  = 0.003) were significantly associated with poor OS, and nerve root sacrifice showed a trend towards significance ( P  = 0.088). In the multivariate models, previous surgery and intralesional resection were significantly related to LR, while increasing age and motor deficit of C or D were associated with poor OS. Conclusions This study identified two predictive variables for LRFS (previous tumor surgery and type of surgical resection) and two for OS (age and impaired motor function) in surgically treated SC patients. Our results indicate that en bloc resection reduces LR but does not influence OS. However, this was likely due to short follow-up (3.2 years).

Local progression of primary skull base chordoma (PSBC) is a sign of treatment failure. Predicting the postoperative progression of PSBC can aid in the development of individualized treatment plans to improve patients’ progression-free survival (PFS) after surgery. This study aimed to develop a multiparametric MRI-based fusion radiomic model (FRM) and clinicoradiomic model (CRM) via radiomic and clinical analysis and to explore their validity in predicting postoperative progression in PSBC patients before surgery. In this retrospective study, a total of 129 patients with PSBC from our institution, including 57 patients with progression, were enrolled and randomized to the training set (TS) or the validation set (VS) at a 2:1 ratio. Radiomic features were extracted and dimensionally reduced from 3.0 T/axial T2-weighted imaging (T2WI), T1-weighted imaging (T1WI) and contrast-enhanced T1-weighted imaging (CE-T1WI) sequences for each patient, and the features were used for radiomic analysis. Univariate and multivariate Cox regression analyses were used to screen for key clinical factors. We constructed models on the basis of multivariate logistic regression analysis. Receiver operating characteristic (ROC) curve, calibration curve, and decision curve analyses were performed to evaluate the performance of the clinical model (CM), FRM and CRM. Through analysis, we found that blood supply was the only significantly different clinical factor in the CM. For the FRM, the area under the receiver operating characteristic curve (AUC) of the TS was 0.925, and the calibration curves were consistent across the TS. In the CRM, the AUC of the TS was 0.929, the calibration curve analysis was consistent for both the TS and the VS, and the DCA showed that the net benefit was greater at a threshold probability of > 0% for both the TS and the VS. Our proposed FRM can help clinicians better predict PSBC progression preoperatively, and the use of the CRM can lead to the development of more appropriate protocols to improve patients’ PFS after surgery.

Purpose The management of chordoma or chondrosarcoma involving the spine is often challenging due to adjacent critical structures and tumor radioresistance. Spine stereotactic radiosurgery (SSRS) has radiobiologic advantages compared with conventional radiotherapy, though there is limited evidence on SSRS in this population. We sought to characterize the long-term local control (LC) of patients treated with SSRS. Methods We retrospectively reviewed patients with chordoma or chondrosarcoma treated with dose-escalated SSRS, defined as 24 Gy in 1 fraction to the gross tumor volume. Overall survival (OS) was calculated by Kaplan-Meier functions. Competing risk analysis using the cause-specific hazard function estimated LC time. Results Fifteen patients, including 12 with chordoma and 3 with chondrosarcoma, with 22 lesions were included. SSRS intent was definitive, single-modality in 95% of cases (N = 21) and post-operative in 1 case (5%). After a median censored follow-up time of 5 years (IQR 4 to 8 years), median LC time was not reached (IQR 8 years to not reached), with LC rates of 100%, 100%, and 90% at 1 year, 2 years, and 5 years. The median OS was 8 years (IQR 3 years to not reached). Late grade 3 toxicity occurred after 23% of treatments (N = 5, fracture), all of which were managed successfully with stabilization. Conclusion Definitive dose-escalated SSRS to 24 Gy in 1 fraction appears to be a safe and effective treatment for achieving durable local control in chordoma or chondrosarcoma involving the spine, and may hold particular importance as a low-morbidity alternative to surgery in selected cases.

Gross total resection (GTR) of cranial base chordomas represents a surgical challenge because of the location, invasiveness, and tumor extension. In the past decade, the endoscopic endonasal approach (EEA) has been used with notable outcomes. To present the endoscopic endonasal experience in the treatment of cranial base chordomas at our institution. From April 2003 to March 2011, 60 patients underwent an EEA for primary (n = 35) or previously treated (n = 25) cranial base chordomas. We evaluated the degree of GTR and complications. We studied the factors that influenced outcomes and compared our surgical results in the early and late years of our experience. The overall rate of GTR of cranial base chordomas was 66.7% (82.9% in primary and 44% in previously treated patients). The most important limitations for GTR were tumor volume greater than 20 cm (P = .042), tumor location in the lower clivus with lateral extension (P = .022), and previously treated disease (P = .002). The learning curve had a significant impact on GTR, increasing the success rate to 88.9% (92.6% in primary patients and 63.6% in previously treated patients) during recent years (P < .0001). The most frequent complication was cerebrospinal fluid leak (20%) resulting in meningitis in 3.3%. Carotid injuries occurred in 2 patients without any resulting deficit. Neurological complications included new cranial neuropathies (6.7%) and long tract deficits (1.7%). There was no operative mortality in our series. For the treatment of cranial base chordomas, the EEA is a competitive alternative to transcranial approaches with minimal morbidity and high success rates of GTR when performed by experienced cranial base surgeons.