Clinical practice guidelines for the treatment of Ewing sarcoma (Spanish Sarcoma Research Group-GEIS).

Ewing sarcoma is a small round-cell sarcoma characterized by gene fusion involving EWSR1 (or another TET family protein like FUS) and an ETS family transcription factor. The estimated incidence of this rare bone tumor, which occurs most frequently in adolescents and young adults, is 0.3 per 100,000/year. Although only 25% of patients with Ewing sarcoma are diagnosed with metastatic disease, historical series show that this is a systemic disease. Patient management requires multimodal therapies-including intensive chemotherapy-in addition to local treatments (surgery and/or radiotherapy). In the recurrent/refractory disease setting, different approaches involving systemic treatments and local therapies are also recommended as well as patient inclusion in clinical trials whenever possible. Because of the complexity of Ewing sarcoma diagnosis and treatment, it should be carried out in specialized centers and treatment plans should be designed upfront by a multidisciplinary tumor board. These guidelines provide recommendations for diagnosis, staging, and multimodal treatment of Ewing sarcoma.

A descriptive study on the adjacent segment degeneration related signs following a lumbar fusion procedure.

BACKGROUND: Adjacent segment degeneration (ASD) is a frequent complication following vertebral fusion procedures and is defined as the condition where patients recover after the initial procedure but develop compatible symptoms with radiological injuries in the segments adjacent to the fused ones at a later stage. The objective of the study was to describe the frequency and analysis of ASD related signs following a lumbar fusion procedure. METHODS: Observational descriptive retrospective study on patients with degenerative or instability conditions, operated on by posterolateral or circumferential lumbar fusion procedure. Pedicle screws, interbody peek cages (polyether-ether-ketone) and autologous bone graft were used. Clinical (pain and disability) and radiological (instability, rotation, disc height loss, radiological degeneration evaluated by X-ray and MR) variables were analysed. RESULTS: Postoperative disc height loss was observed in 159 free discs among 112 patients (42.6%) (95% CI: 36.4-48.8%). Anterior or posterior slippage (anterolisthesis or retrolisthesis) at the end of the follow-up period was observed in 33 patients (12.5%). Upper segment rotation increased in the postoperative period in 36 patients (13.6%). Radiological disc degeneration was observed in 107 discs among 72 patients, being more frequent in the immediate upper disc with grade 2 and 3 changes at the end of follow-up in 48 discs from 35 patients (13.6%) (95% CI: 13.4-23.1%). Radiological ASD signs were observed in 151 patients (57.4%; 95% CI: 51.2-63.6%) and 53 of them (20.2%; 95% CI: 15.1-25.2%) who also showed clinical ASD symptoms (clinical and radiological ASD). Degeneration changes with degrees IV and V shown by a preoperative and magnetic resonance (MR) study at end of the follow-up period performed in 73 patients (27.7%), were observed in 46 discs among 32 patients (43.8%) (95% CI: 31.8-55.9%). CONCLUSIONS: Radiological ASD signs evaluated in every free disc following a lumbar fusion procedure are observed with a variable frequency. All free discs after fusion were assessed as they could indicate mechanisms of compensation of lordosis loss and should be taken into consideration in a prospective revision surgery.

Risk factors related to adjacent segment degeneration: retrospective observational cohort study and survivorship analysis of adjacent unfused segments.

OBJECT: Adjacent segment disease (ASD) has been described as a frequent complication after a lumbar spinal fusion procedure, though its incidence and the factors related to its appearance are not well established. The radiographic signs that identify ASD in unfused segments may be a consequence of biomechanical changes induced by the fusion procedure. This study sought to analyse the incidence of radiographic changes (radiographic ASD) in all adjacent unfused segments, the clinical changes that require a second procedure (clinical ASD), and the risk factors of their appearance evaluated at different follow-up times. METHODS: We conducted a retrospective cohort study of patients fused for degenerative spine disease and instability to analyse ASD risk factors using actuarial estimation, comparison of the Kaplan-Meier survival curves of each variable, and Cox proportional-hazards regression analysis. RESULTS: Among the 263 patients included in the study, radiographic changes were observed in 57.4% and related clinical changes in 20.2%. The univariate analysis showed a higher risk of ASD in patients with smaller post- vs. pre-operative lumbar lordosis (p = .018), diagnosis of lumbar canal stenosis (p = .019), fusion of three or more vs. fewer levels (p = .009) and those fixed with top-loading screws vs. side-connecting screws (p = .001). Cox proportional-hazards regression analysis showed that the use of top-loading pedicle screws and three or more levels of fusion led to a 3- and 2-fold higher risk of degeneration in adjacent unfused segments respectively. CONCLUSIONS: Risk of a second surgical procedure due to clinical changes is 3-fold higher in patients with three or more levels of fusion, and 2.5-fold higher in patients intervened with top-loading pedicle screws.

Prediction Accuracy of Common Prognostic Scoring Systems for Metastatic Spine Disease: Results of a Prospective International Multicentre Study of 1469 Patients.

STUDY DESIGN: A prospective multicenter cohort study. OBJECTIVE: To assess the clinical accuracy of six commonly cited prognostic scoring systems for patients with spinal metastases. SUMMARY OF BACKGROUND DATA: There are presently several available methods for the estimation of prognosis in metastatic spinal disease, but none are universally accepted by surgeons for clinical use. These scoring systems have not been rigorously tested and validated in large datasets to see if they are reliable enough to inform day-to-day patient management decisions. We tested these scoring systems in a large cohort of patients. A total of 1469 patients were recruited into a secure internet database, and prospectively collected data were analyzed to assess the accuracy of published prognostic scoring systems. METHODS: We assessed six prognostic scoring systems, described by the first authors Tomita, Tokuhashi, Bauer, van der Linden, Rades, and Bollen. Kaplan-Meier survival estimates were created for different patient subgroups as described in the original publications. Harrell's C-statistic was calculated for the survival estimates, to assess the concordance between estimated and actual survival. RESULTS: All the prognostic scoring systems tested were able to categorize patients into separate prognostic groups with different overall survivals. However none of the scores were able to achieve "good concordance" as assessed by Harrell's C-statistic. The score of Bollen and colleagues was found to be the most accurate, with a Harrell's C-statistic of 0.66. CONCLUSION: No prognostic scoring system was found to have a good predictive value. The scores of Bollen and Tomita were the most effective with Harrell's C-statistic of 0.66 and 0.65, respectively. Prognostic scoring systems are calculated using data from previous years, and are subject to inaccuracies as treatments advance in the interim. We suggest that other methods of assessing prognosis should be explored, such as prognostic risk calculation. LEVEL OF EVIDENCE: 3.

The impact of urgent intervention on the neurologic recovery in patients with thoracolumbar fractures.

BACKGROUND: We carried out an observational longitudinal retrospective study between 2000 and 2009 in 28 patients who underwent surgery for unstable vertebral fractures with neurologic deficits. METHODS: For the statistical analysis, we used the Chi2-test to compare proportions in independent groups and the exact Fisher test and the Wilcoxon test for repeated measures, and we compared the mean values using the Mann-Whitney U test at a significance level of P<0.05. Timing to surgical intervention (urgent ≤8 vs. >8 h), and neurologic status using the American Spinal Injury Association (ASIA) Impairment Scale at baseline and at the end of follow-up were assessed. We tested the ASIA score improvement at the end of follow-up using multiple regression analysis, adjusted by variables such as ISS, timing of intervention, location, approach and type of fracture. RESULTS: Twenty-eight patients were included in the analysis. Of the total, 11 (39.2%) underwent surgery urgently (≤8 h) and 17 (60.8%) in >8 h. The mean difference in the neurologic improvement in all patients was 0.97 (95% CI, 0.51-1.42) and was statistically significant (P=0.001). The mean difference in the neurologic improvement in patients with incomplete lesions was 1.59 (95% CI, 1.01-2.17, P=0.001). In these patients, the mean improvement for those intervened in less than 8 h was 1.73 compared to those operated on after more than 8 h (mean improve 0.47) with a difference statistically significant (P=0.007). CONCLUSIONS: Urgent surgery was associated with neurologic improvement in patients with spinal cord injury (SCI). This improvement was mainly observed in patients with an incomplete lesion.

Metastatic Spine Tumor Epidemiology: Comparison of Trends in Surgery Across Two Decades and Three Continents.

BACKGROUND: Indications for surgery for symptomatic spinal metastases have become better defined in recent years, and suitable outcome measures have been established against a changing backdrop of patient characteristics, tumor behavior, and oncologic treatments. Nonetheless, variations still exist in the local management of patients with spinal metastases. In this study, we aimed to review global trends and habits in the surgical treatment of symptomatic spinal metastases, and to examine how these have changed over the last 25 years. METHODS: In this cohort study of consecutive patients undergoing surgery for symptomatic spinal metastases, data were collected using a secure Internet database from 22 centers across 3 continents. All patients were invited to participate in the study, except those unable or unwilling to give consent. RESULTS: There was a higher incidence of colonic, liver, and lung carcinoma metastases in Asian countries, and more frequent presentation of breast, prostate, melanoma metastases in the West. Trends in surgical technique were broadly similar across the centers. Overall survival rates after surgery were 53% at 1 year, 31% at 2 years, and 10% at 5 years after surgery (standard error 0.013 for all). Survival improved over successive time periods, with longer survival in patients who underwent surgery in 2011-2016 compared with those who underwent surgery in earlier time periods. CONCLUSIONS: Surgical habits have been fairly consistent among countries worldwide and over time. However, patient survival has improved in later years, perhaps due to medical advances in the treatment of cancer, improved patient selection, and operating earlier in the course of disease.

Lumbar disc herniation surgery with microdiscectomy plus interspinous stabilization: Good clinical results, but failure to lower the incidence of re-operation.

BACKGROUND: Discectomy is sometimes associated with recurrence of disc herniation and pain after surgery. The evidence to use an interspinous dynamic stabilization system (IDSS) in association with disc excision to prevent pain and re-operation, remains controversial. METHODS: Patients (age 18-50 years) presenting with lumbago/sciatica (ICD-10-CM M54.3, M54.4) due to voluminous lumbar disc herniation were eligible for participation. Patients underwent microdiscectomy plus IDSS. The primary outcome measure was the clinical efficacy using Owestry disability index(ODI) and visual analogue pain scale (VAS). We also evaluated several other outcome parameters including: length of stay and costs during hospital admission, 90-day complication rate, and 1-year re-operation rate. This prospective observational study was carried out from January 2015 to August 2016. RESULTS: A total of 30 patients whose mean age was 38.6(±9.2) years were included. ODI score dropped from 62.93(±16.45) to 13.50(±16.67), representing 78.54% (95% C.I 68.07-88.66%) improvement of the baseline score after one year (p < 0.001). Patients had 90 day re-admission and 1 year re-operation rates of 4/30(13.3%) and 3/30(10%) respectively. Length of stay was 2.1 ±â€¯1.2 days. In-Hospital cost was 1069.8 ±â€¯288.4 € (not including 1500€ of the implant). Implant related complications were common 12/30(40%), although they did not have any clinical consequences. CONCLUSION: Our short-term experience indicates that microdiscectomy plus interspinous device is safe and it shows good clinical results, although the clinical improvement seems to be due to microdiscectomy, without the implant adding any extra benefit. The addition of IDSS did not protect against re-operation, and it increased the surgical expenses.

Length of stay, costs, and complications in lumbar disc herniation surgery by standard PLIF versus a new dynamic interspinous stabilization technique.

BACKGROUND: The number of lumbar spine surgeries has been increasing during the last 20 years, which also leads to an increase in hospital costs and complications related to surgery. Therefore, there is a greater concern about the costs and safety of the techniques and implants used. METHODS: Patients (aged from 18 to 50 years) presenting with lumbago /sciatica (ICD-10-CM M54.3, M54.4) due to lumbar disc herniation lasting more than 12 weeks, were included. Patients with disc herniation larger than size-2 or size-3 according to the MSU Classification were eligible for participation. Intervention was divided in two groups. In Group 1, patients underwent microdiscectomy and Interspinous Dynamic Stabilization System (IDSS). Meanwhile, in Group 2, patients received discectomy and posterior lumbar interbody fusion (PLIF). The primary outcome measure was the length of stay and costs during hospital admission. We also evaluated several other outcome parameters, including 90- day readmission rate, 90-day complication rate, and re-operations rate. The study was an observational prospective cohort study carried out from January 2015 to August 2016 in which two surgical techniques were compared. Our hypothesis was that a less aggressive procedure, such as discectomy and DSS, will decrease the length of stay and costs, and that it will also reduce the rate of complications with respect to PLIF. RESULTS: A total of 67 patients (mean age 39.8 ± 8.4 years) were included. Patients in the PLIF group had a length of stay increase of 109% (4.52 ± 1.76 days vs 2.16 ± 1.18 days p < 0.001) and an in-hospital cost increase of 71% (1821.97 ± 460.41€ vs. 1066.20 ± 284.34€ p < 0.001). The reduction of one day of stay is equivalent to a reduction of total in-hospital costs of 12.5%. Patients in the IDSS cohort had no significant differences regarding PLIF cohort in the 90-day readmission rate (12.9% vs 11.1% € p > 0.999, respectively), 90-day re-operation rate (12.9% vs 11.1% € p > 0.999) and 90-day complication rates (35.5% vs 52.8% € p > 0.156). Dural tear and urinary tract infection rates were higher in the PLIF cohort (13.9% vs 3.2%. p = 0.205 and 11.1% vs 0% p = 0.118, respectively). Implant related complications were the most frequent in both IDSS and PLIF groups (32.3% vs 38.9% p = 0.572). CONCLUSIONS: Patients who underwent IDSS had a significant decrease of the length of stay and costs in relation to PLIF group. No significant differences were found in 90-day readmission and reintervention rates for both groups. Although differences were not significant, dural tear and urinary tract infection rates were lower in the interspinous group. IDSS or PLIF after discectomy, did not protect against subsequent 90-day re-operation or readmission compared to discectomy alone.

Free disease long-term survival in primary thoracic spine leiomyosarcoma after total en bloc spondylectomy: A case report.

INTRODUCTION: To describe an unusual primary vertebral leiomyosarcoma in thoracic spine. PRESENTATION OF CASE: An isolated lesion of the T11 vertebra in a 62-year-old woman with no neurologic deficit is reported. Imaging findings indicated a nonspecific high-grade malignant lesion. TC-guided biopsy failed thus open incisional biopsy was needed. A diagnosis of low-intermediate mesenchymal sarcoma was made. A total en bloc spondylectomy of T11 was performed with three-column reconstruction. The histology and immunostaining showed the appearance of leiomyosarcoma. After diagnosis, post-operative radiation therapy was performed. Metastatic lesion was ruled out by CT scans of the chest, abdomen and pelvis, in addition to total body radionuclide scanning and 18-F-FDG-PET. After five years of follow-up, no signs of local recurrence, metastasis or distant lesions suggesting a primary lesion were observed. DISCUSSION: Vertebral primary leiomyosarcoma is exceedingly rare. Primary vertebral leiomyosarcoma diagnosis must be performed when the metastatic origin is excluded. For the treatment of primary tumors, total en bloc spondylectomy (TES) is the technique of choice to achieve marginal or wide tumor resection, decrease the risk of local recurrence and remote lesions and increase survival. CONCLUSIONS: A well-planned pre-operative study and a wide surgical excision can result in local tumor control and long-term survival. This case presents the longest disease-free survival period of a primary leiomyosarcoma in spinal location after total en bloc spondylectomy.

Characteristics of Patients Who Survived < 3 Months or > 2 Years After Surgery for Spinal Metastases: Can We Avoid Inappropriate Patient Selection?

PURPOSE: Survival after metastatic cancer has improved at the cost of increased presentation with metastatic spinal disease. For patients with pathologic spinal fractures and/or spinal cord compression, surgical intervention may relieve pain and improve quality of life. Surgery is generally considered to be inappropriate if anticipated survival is < 3 months. The aim of this international multicenter study was to analyze data from patients who died within 3 months or 2 years after surgery, to identify preoperative factors associated with poor or good survival, and to avoid inappropriate selection of patients for surgery in the future. PATIENTS AND METHODS: A total of 1,266 patients underwent surgery for impending pathologic fractures and/or neurologic deficits and were prospectively observed. Data collected included tumor characteristics, preoperative fitness (American Society of Anesthesiologists advisory [ASA]), neurologic status (Frankel scale), performance (Karnofsky performance score [KPS]), and quality of life (EuroQol five-dimensions questionnaire [EQ-5D]). Outcomes were survival at 3 months and 2 years postsurgery. Univariable and multivariable logistic regression analyses were used to find preoperative factors associated with short-term and long-term survival. RESULTS: In univariable analysis, age, emergency surgery, KPS, EQ-5D, ASA, Frankel, and Tokuhashi/Tomita scores were significantly associated with short survival. In multivariable analysis, KPS and age were significantly associated with short survival (odds ratio [OR], 1.36; 95% CI, 1.15 to 1.62; and OR, 1.14; 95% CI, 1.02 to 1.27, respectively). Associated with longer survival in univariable analysis were age, number of levels included in surgery, KPS, EQ-5D, Frankel, and Tokuhashi/Tomita scores. In multivariable analysis, the number of levels included in surgery (OR, 1.21; 95% CI, 1.06 to 1.38) and primary tumor type were significantly associated with longer survival. CONCLUSION: Poor performance status at presentation is the strongest indicator of poor short-term survival, whereas low disease load and favorable tumor histology are associated with longer-term survival.