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OBJECTIVE Intramedullary spinal cord tumors (IMSCTs) are typically treated with maximal safe resection, during which neurosurgeons often monitor for neurological injury using muscle motor evoked potential (mMEP) and direct wave (D-wave) neuromonitoring. The predictive value of changes in D-waves for identifying motor outcomes is underexplored. This study evaluated the utility of D-waves for predicting postoperative motor deficits. METHODS Patients who underwent resection of a primary IMSCT with mMEP neuromonitoring from 2003 to 2023 at a tertiary care hospital were identified. Patients who underwent D-wave monitoring in addition to mMEP monitoring were compared to those who underwent mMEP monitoring alone using the Mann-Whitney U-test, chi-square test, and Fisher’s exact test. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of D-wave and mMEP monitoring for identifying new neurological deficits immediately postoperatively and at 1 month, 6 months, and last follow-up were calculated. RESULTS After matching, 125 patients were included (median age 42.0 years; 57.6% male; median follow-up 34.0 months), of whom 88 had both mMEP and D-wave data. The most common pathologies were ependymoma (64.0%) and astrocytoma (17.6%). Patients who did and did not undergo D-wave neuromonitoring had similar preoperative neurological function, primary pathology, tumor grade, and tumor location. D-wave use was associated with increased gross-total resection (88.6% vs 64.9%, p = 0.002) and reduced mortality (5.7% vs 24.3%, p = 0.007), length of stay (5.0 vs 6.0 days, p = 0.033), and 30-day readmission (2.3% vs 13.5%, p = 0.013) and reoperation (1.1% vs 10.8%, p = 0.012). At the 6-month follow-up, D-wave monitoring alone was superior to mMEP and combination monitoring for detecting new motor deficits. D-wave monitoring had peak sensitivity (77.8%) and NPV (96.5%) at 6 months and peak specificity (95.8%) and PPV (76.9%) in the immediate postoperative period. CONCLUSIONS D-wave monitoring was associated with reduced mortality and was more accurate than mMEP monitoring alone or combination monitoring for detecting new postoperative neurological deficits. Further prospective studies are needed to validate these results.

OBJECTIVE Management of locally invasive colorectal carcinoma at any stage currently involves surgical excision followed by chemoradiotherapy; however, the prognosis is poor, with a 5-year overall survival (OS) of only 5%. Failure to achieve gross-total resection is associated with poorer OS, and patients with residual tumor postresection (R1 or R2 resection) have a median OS of 7 months compared with 23 months in those who undergo resection with negative margins (R0 resection). For tumors that have invaded the sacrum, sacrectomy becomes necessary to achieve R0 resection. The objective of this study was to provide a descriptive multicenter account of resection for locally invasive colorectal carcinoma with sacral invasion, focusing on the association of tumor morphometry with surgical planning and perioperative outcomes. METHODS Demographic, comorbidity, clinical, tumor-specific, operative characteristic, and outcome data were collected on all patients who underwent resection of colorectal carcinoma with concurrent sacral resection between January 2005 and May 2022. Patients were grouped into those having undergone surgery for purely palliative intent, or those with resection with attempt at local control and dichotomized into level of osteotomy (either proximal or distal to the S2–3 level). RESULTS Twenty-two patients (median age 50.5 [IQR 43.3–60.0] years, 54.5% female) underwent sacrectomy for colorectal carcinoma. Operative records indicated intent for local control in 14 patients and palliative in the remaining 8 patients. Palliative surgical intent was based primarily on the presence of distant metastases. There was no significant difference in median local progression-free survival between patients undergoing osteotomy proximal to the S2–3 level and those undergoing osteotomy distal to the S2–3 level. CONCLUSIONS En bloc resection is believed to offer the best local control in patients with locally invasive colorectal carcinoma. The present descriptive series highlights outcomes of en bloc resection with partial or full sacrectomy in patients with tumors showing local extension into the sacrum. Complications are common, most often in the form of wound dehiscence or infection, and many patients require placement in a rehabilitation or intermediate-care facility upon discharge. However, for those with stage III (locally aggressive) disease, median OS exceeds 16 years, suggesting that such aggressive management with en bloc resection may be warranted in properly selected patients.

OBJECTIVE Intramedullary spinal cord tumors (IMSCTs) are rare neoplasms whose treatment is often technically challenging. Given the low volume seen at most centers, perioperative outcomes have been reported infrequently. Here, the authors present the largest single-institution series of IMSCTs, focusing on the clinical presentation, histological makeup, perioperative outcomes, and long-term survival of surgically treated patients. METHODS A cohort of patients operated on for primary IMSCTs at a comprehensive cancer center between June 2002 and May 2020 was retrospectively identified. Data on patient demographics, tumor histology, neuraxial location, baseline neurological status, functional deficits, and operative characteristics were collected. Perioperative outcomes of interest included length of stay, postoperative complications, readmission, reoperation, and discharge disposition. Data were compared across tumor histologies using the Kruskal-Wallis H test, chi-square test, and Fisher exact test. Pairwise comparisons were conducted using Tukey’s honest significant difference test, chi-square test, and Fisher exact test. Long-term survival was assessed across tumor categories and histological subtype using the log-rank test. RESULTS Three hundred two patients were included in the study (mean age 34.9 ± 19 years, 77% white, 57% male). The most common tumors were ependymomas (47%), astrocytomas (31%), and hemangioblastomas (11%). Ependymomas and hemangioblastomas disproportionately localized to the cervical cord (54% and 59%, respectively), whereas astrocytomas were distributed almost equally between the cervical cord (36%) and thoracic cord (38%). Clinical presentation, extent of functional dependence, and postoperative 30-day outcomes were largely independent of underlying tumor pathology, although tumors of the thoracic cord had worse American Spinal Injury Association (ASIA) grades than cervical tumors. Rates of gross-total resection were lower for astrocytomas than for ependymomas (54% vs 84%, p < 0.01) and hemangioblastomas (54% vs 100%, p < 0.01). Additionally, 30-day readmission rates were significantly higher for astrocytomas than ependymomas (14% vs 6%, p = 0.02). Overall survival was significantly affected by the underlying pathology, with astrocytomas having poorer associated prognoses (40% at 15 years) than ependymomas (81%) and hemangioblastomas (66%; p < 0.01) and patients with high-grade ependymomas and astrocytomas having poorer long-term survival than those with low-grade lesions (p < 0.01). CONCLUSIONS The neuraxial location of IMSCTs, extent of resection, and postoperative survival differed significantly across tumor pathologies. However, perioperative outcomes did not vary significantly across tumor cohorts, suggesting that operative details, rather than pathology, may have a stronger influence on the short-term clinical course, whereas pathology appears to have a stronger impact on long-term survival.

Abstract Background Chordomas are the most common primary spinal column malignancy in the United States. The aim of this study was to determine whether chordomas may be detected by evaluating mutations in circulating tumor DNA (ctDNA). Methods Thirty-two patients with a biopsy-confirmed diagnosis of chordoma had blood drawn pre-operatively and/or at follow-up appointments. Mutations in the primary tumor were identified by whole exome sequencing and liquid biopsy by ddPCR and/or RACE-Seq was used to detect one or more of these mutations in plasma ctDNA at concurrent or later time points. Results At the time of initial blood draw, 87.1% of patients were ctDNA positive (P &lt;.001). Follow-up blood draws in twenty of the patients suggest that ctDNA levels may reflect the clinical status of the disease. Patients with positive ctDNA levels were more likely to have greater mutant allele frequencies in their primary tumors (P = .004) and undergo radiotherapy (P = .02), and the presence of ctDNA may correlate with response to systemic chemotherapy and/or disease recurrence. Conclusions Detection of ctDNA mutations may allow for the detection and monitoring of disease progression for chordomas.

Abstract Background Spinal chordomas, a subtype of primary spinal column malignancies (PSCM), are rare tumors with poor prognosis, and we have limited understanding of the molecular drivers of neoplasia. Methods Study design was a retrospective review of prospectively collected data with cross-sectional survival. Archived paraffin embedded pathologic specimens were collected for 133 patients from 6 centers within Europe and North America between 1987 and 2012. Tumor DNA was extracted and the human telomerase reverse transcriptase (hTERT) promoter was sequenced. The hTERT mutational status was correlated with overall survival (OS) and time to first local recurrence. Results Ninety-two chordomas, 26 chondrosarcomas, 7 osteosarcomas, 3 Ewing’s sarcomas, and 5 other malignant spinal tumors were analyzed. Median OS following surgery was 5.8 years (95% CI: 4.6 to 6.9) and median time to first local recurrence was 3.9 years (95% CI: 2.5 to 6.7). Eight chordomas, 2 chondrosarcomas, 1 Ewing’s sarcoma, and 1 other malignant spinal tumor harbored either a C228T or C250T mutation in the hTERT promoter. In the overall cohort, all patients with hTERT mutation were alive at 10 years postoperative with a median OS of 5.1 years (95% CI: 4.5 to 6.6) (P = 0.03). hTERT promoter mutation was observed in 8.7% of spinal chordomas, and 100% of chordoma patients harboring the mutation were alive at 10 years postoperative compared with 67% patients without the mutation (P = 0.05). Conclusions We report for the first time that hTERT promoter mutations C228T and C250T are present in approximately 8.7% of spinal chordomas. The presence of hTERT mutations conferred a survival benefit and could potentially be a valuable positive prognostic molecular marker in spinal chordomas.

Study Design: This is a retrospective study. Objective: To report the safety profile of S2-alar-iliac (S2AI) in patients over 60, comparing S2AI screws with iliac screws (ISs). Summary of Background Data: The surgical management involving the lumbosacropelvic spine remains a challenge due to high mechanical demand and risk of pseudarthrosis. Previous articles showed lower rates of complications in patients receiving S2AI screws than ISs; however, none of them have focused on patients aged over 60 who may harbor significant comorbidities and thus require more meticulous perioperative management, given these invasive and lengthy procedures. Materials and Methods: Retrospective review of clinical records from 2010 to 2015 identified 60 patients undergoing lumbosacropelvic fixation (17 patients with ISs and 43 patients with S2AI screws) who satisfied the following criteria: (1) patients aged over 60 years old and (2) patients with >1-year follow-up periods. Rates of complications such as unplanned reoperation and cardiorespiratory complications were collected and statistically analyzed. Results: Baseline characteristics such as age, sex, and comorbidities were similar in both groups. The S2AI group had lower rates of reoperation (18.6% vs. 47.4%; P=0.02), surgical site infection (2.3% vs. 29.4%; P=0.006), wound dehiscence (2.3% vs. 29.4%; P=0.006), and postoperative anemia (7.0% vs. 29.4%; P=0.03) and had lower total volume of estimated blood loss (EBL) (mL) (1846.4 vs. 2721.2; P=0.02) and transfused red blood cell units (7.2 vs. 4.7; P=0.04) than the IS group, while rates of L5–S1 pseudarthrosis and other cardiorespiratory complications were similar in both groups. In multivariate analysis, operative time, body mass index, and use of S2AI screws over ISs were independent predictors of EBL. Conclusions: Use of S2AI screws over ISs in patients aged over 60 was associated with lower rates of reoperation, surgical site infection, wound dehiscence, and lower volume of EBL and red blood cell transfusion and is a viable surgical option.

Study Design: Systematic review. Objectives: We conducted a systematic review of the literature to answer the following questions regarding the use of steroid therapy in metastatic spinal cord compression (MSCC): 1. In cases of MSCC, what is the effect of steroid administration before definitive radiotherapy or surgery on ambulatory status, bowel and bladder function and survival? 2. What steroid dosing regimens are associated with the best outcomes concerning neurological symptoms and complication prevention in cases of MSCC? Summary of Background Data: Currently, there is significant variation in the initial bolus dose, daily maintenance dose and duration of treatment when steroids are used as a bridge to definitive therapy for MSCC. Methods: A literature search following PRISMA guidelines was conducted in June 2016, using Medline via Ovid SP, Medline via PubMed, Embase, Biosis Previews and the Cochrane Library. Search terms used in each database varied slightly to optimize results. All generic steroid formulations were included along with spinal cord compression or myelopathy combined with metastatic or malignant tumors. Papers discussing acute traumatic causes of spinal cord compression were excluded, as were papers discussing cord compression from nonmetastatic tumors or epidural lipomatosis. Subjects were limited to adult humans undergoing definitive treatment with radiotherapy or surgery. Results: Of the 309 papers retrieved, 66 full text studies were reviewed and 6 papers were found to address the stated questions. Conclusions: There is a paucity of high quality literature evaluating the use of steroids in MSCC. On the basis of the evidence available an initial 10 mg intravenous bolus of dexamethasone followed by 16 mg PO QD has been associated with fewer complications compared with 100 mg bolus and 96 mg QD. Weaning of steroids should occur rapidly after definitive treatment. Risk of gastric bleeding or perforation can be managed with the routine use of proton-pump inhibitors. Level of Evidence: Level IIIa.

Abstract BACKGROUND Surgical treatment of severe cervicothoracic kyphotic deformity may require the use of 3-column osteotomies such as the pedicle subtraction osteotomy and vertebral column resection (VCR), or VCR with anterior longitudinal ligament resection. Such procedures are extensive and are associated with high intra- and perioperative morbidity, in part, due to the need for risky reduction maneuvers. OBJECTIVE To describe a novel technique utilizing a laterally placed articulating hinge to facilitate kyphotic deformity correction of the cervicothoracic spine. METHODS A patient with severe chin-on-chest deformity of the cervicothoracic spine presented for evaluation and a 2-stage VCR with anterior longitudinal ligament resection was planned. To reduce the risk of intraoperative neurological injury and for increased control during reduction maneuvers, lateral instrumentation was placed through the chest wall resection above and below the level of VCR, which was adjoined with an articulating hinge rod apparatus. RESULTS Satisfactory reduction of the kyphosis was achieved utilizing the hinge rod apparatus for controlled deformity correction. The patient remained neurologically intact following this procedure with improvement in their spinal alignment. CONCLUSION We present a novel technique utilizing a lateral hinge rod apparatus for efficient, controlled correction of severe kyphotic deformity.

OBJECTIVE The purpose of this study was to report the long-term clinical outcomes following 3- and 4-level anterior cervical discectomy and fusion (ACDF). METHODS A retrospective review of all adult neurosurgical patients undergoing elective ACDF for degenerative disease at a single institution between 1996 and 2013 was performed. Patients who underwent first-time 3- or 4-level ACDF were included; patients with previous cervical spine surgery, those undergoing anterior/posterior approaches, and those with corpectomy were excluded. Outcome measures included perioperative complication rates, fusion rates, need for revision surgery, Nurick Scores, Odom's criteria, symptom resolution, neck visual analog scale (VAS) pain score, and persistent narcotics usage. RESULTS Seventy-one patients who underwent 3-level ACDF and 26 patients who underwent 4-level ACDF were identified and followed for an average of 7.6 ± 4.2 years. There was 1 case (3.9%) of deep wound infection in the 4-level group and 1 case in the 3-level group (1.4%; p = 0.454). Postoperatively, 31% of patients in the 4-level group complained of dysphagia, compared with 12.7% in the 3-level group (p = 0.038). The fusion rate was 84.6% after 4-level ACDF and 94.4% after 3-level ACDF (p = 0.122). At last follow-up, a significantly higher proportion of patients in the 4-level group continued to have axial neck pain (53.8%) than in the 3-level group (31%; p = 0.039); the daily oral morphine equivalent dose was significantly higher in the 4-level group (143 ± 97 mg/day) than in the 3-level group (25 ± 10 mg/day; p = 0.030). Outcomes based on Odom's criteria were also different between cohorts (p = 0.044), with a significantly lower proportion of patients in the 4-level ACDF group experiencing an excellent/good outcome. CONCLUSIONS In this study, patients who underwent 4-level ACDF had significantly higher rates of dysphagia, postoperative neck pain, and postoperative narcotic usage when compared with patients who underwent 3-level ACDF. Pseudarthrosis and deep wound infection rates were also higher in the 4-level group, although this did not reach statistical significance. Additionally, a smaller proportion of patients achieved a good/excellent outcome in the 4-level group than in the 3-level group. These findings suggest a significant increase of perioperative morbidity and worsened outcomes for patients who undergo 4- versus 3-level ACDF.

OBJECT A chordoma is an indolent primary spinal tumor that has devastating effects on the patient's life. These lesions are chemoresistant, resistant to conventional radiotherapy, and moderately sensitive to proton therapy; however, en bloc resection remains the preferred treatment for optimizing patient outcomes. While multiple small and largely retrospective studies have investigated the outcomes following en bloc resection of chordomas in the sacrum, there have been few large-scale studies on patients with chordomas of the mobile spine. The goal of this study was to review the outcomes of surgically treated patients with mobile spine chordomas at multiple international centers with respect to local recurrence and survival. This multiinstitutional retrospective study collected data between 1988 and 2012 about prognosis-predicting factors, including various clinical characteristics and surgical techniques for mobile spine chordoma. Tumors were classified according to the Enneking principles and analyzed in 2 treatment cohorts: Enneking-appropriate (EA) and Enneking-inappropriate (EI) cohorts. Patients were categorized as EA when the final pathological assessment of the margin matched the Enneking recommendation; otherwise, they were categorized as EI. METHODS Descriptive statistics were used to summarize the data (Student t-test, chi-square, and Fisher exact tests). Recurrence and survival data were analyzed using Kaplan-Meier survival curves, log-rank tests, and multivariate Cox proportional hazard modeling. RESULTS A total of 166 patients (55 female and 111 male patients) with mobile spine chordoma were included. The median patient follow-up was 2.6 years (range 1 day to 22.5 years). Fifty-eight (41%) patients were EA and 84 (59%) patients were EI. The type of biopsy (p < 0.001), spinal location (p = 0.018), and if the patient received adjuvant therapy (p < 0.001) were significantly different between the 2 cohorts. Overall, 58 (35%) patients developed local recurrence and 57 (34%) patients died. Median survival was 7.0 years postoperative: 8.4 years postoperative for EA patients and 6.4 years postoperative for EI patients (p = 0.023). The multivariate analysis showed that the EI cohort was significantly associated with an increased risk of local recurrence in comparison with the EA cohort (HR 7.02; 95% CI 2.96–16.6; p < 0.001), although no significant difference in survival was observed. CONCLUSIONS EA resection plays a major role in decreasing the risk for local recurrence in patients with chordoma of the mobile spine.