Search Results (55)

Background: Primary spinal gliomas are rare in the pediatric population. Separately, FGFR1 genomic aberrations are also uncommon in spinal cord tumors. We report a case of a previously well adolescent who presented with progressive symptoms secondary to an intramedullary tumor with unique radiological and molecular characteristics. Case Presentation: A previously well 17-year-old male presented with worsening mid-back pain associated with lower limb long-tract signs. Magnetic resonance imaging (MRI) of his neuro-axis reported a long-segment intramedullary lesion with enhancing foci and a multi-septate syrinx containing hemorrhagic components from C4 to T12. The largest enhancement focus was centered at T7. Additional MRI sequences observed no intracranial involvement or vascular anomaly. He underwent an emergent laminoplasty and excision of the thoracic lesion. Intraoperative findings demonstrated a soft, grayish intramedullary tumor associated with extensive hematomyelia that had multiple septations. Active fenestration of the latter revealed blood products in various stages of resolution. Postoperatively, the patient recovered well, with neurological improvement. Final histology reported a circumscribed low-grade glial neoplasm. Further molecular interrogation via next-generation sequencing panels showed FGFR1 p.K656E and V561M alterations. The unique features of this case are presented and discussed in corroboration with a focused literature review. Conclusions: We highlight an interesting case of an intramedullary tumor with unusual radiological and pathological findings. Emphasis is on the importance of tissue sampling in corroboration with genomic investigations to guide clinical management. Full article

Primary spinal cord tumors are rare neoplasms representing 2–4% of central nervous system tumors. Despite their low incidence, their impact on neurological function is profound. Historically, tumor classification and management have relied primarily on histopathology. However, advances in molecular diagnostics have highlighted the critical role of genetic alterations in tumor behavior, prognosis, and treatment response. This narrative review summarizes current evidence on genetic mutations in primary intramedullary spinal cord tumors, focusing on their prognostic value and implications for clinical management. Emphasis is placed on the integration of genetic features into diagnostic criteria and clinical practice, as distinct molecular profiles define many spinal cord tumor subtypes. Integration of molecular diagnostics into spinal cord tumor management represents a paradigm shift from morphology-based to biology-driven practice. Genetic alterations inform prognosis, refine risk stratification, and increasingly guide therapeutic decision-making, including the use of targeted therapies and adjuvant radiation. Despite progress, challenges remain due to the rarity of these tumors, small sample sizes, and limited access to molecular testing. Ultimately, molecular precision promises to enhance survival and quality of life for patients with these rare but impactful tumors. Full article

Background: Benign bone tumors and tumor-like lesions in the proximal femur increase the risk of pathological fractures, often requiring surgical intervention. However, no consensus exists on the optimal treatment strategy. We developed a structured approach to guide the selection of implant types (compression hip screw [CHS] or intramedullary nail [IMN]) with or without bone grafting. This study aims to validate our treatment strategy through a retrospective analysis and a review of previous surgical outcomes. Methods: We sought to validate this strategy through a retrospective analysis of 16 patients (6 males and 10 females, mean age at surgery 37.4 years [range, 16–64 years]) with primary benign bone tumors or tumor-like conditions of the proximal femur, including the femoral head and neck. Curettage and synthetic or autologous bone graft was performed according to our treatment flowchart, utilizing either CHS or IMN for internal fixation. We compared the blood loss, operative time, time to full weight bearing, and perioperative complications between the CHS and IMN groups. Results: Blood loss did not significantly differ between the CHS and IMN groups (p = 0.11), but the operative time was significantly longer in the CHS group (p < 0.01). Two CHS cases experienced local recurrence, while no postoperative fractures were observed in either group. The median time to full weight bearing was 5 weeks, consistent with previous reports. No perioperative complications were noted. Conclusions: Our strategy achieved favorable clinical outcomes. IMN was selectively used in patients with non-aggressive benign tumors not involving the femoral head and neck, yielding good results with reduced surgical invasiveness, while in those patients with aggressive disease involving the head and neck, CHS was more appropriate. This approach may serve as a practical guide for surgical decision-making in benign proximal femoral bone tumors. Full article

The aim of this work is to analyse the effectiveness of the medical use of the Cavitron Ultrasonic Surgical Aspirator (CUSA) in microsurgical treatment of Intramedullary Spinal Cord Tumors (IMSCTs), with a focus on the thermo-mechanical effects on neighbouring tissues to assess any potential damage. Indeed, CUSA emerges as an innovative solution, minimally invasive tumor excision technique, enabling controlled and focused operations. This study employs a Finite Element Analysis (FEA) to simulate the vibratory and thermal interactions occurring during CUSA application. A computational model of a vertebral column segment affected by an IMSCT was developed and analysed using ANSYS 2024 software. The simulations examined strain distribution, heat generation, and temperature propagation within the biological tissues. The FEA results demonstrate that the vibratory-induced strain remains highly localised to the application site, and thermal effects, though measurable, do not exceed the critical safety threshold of 46 °C established in the literature. These findings suggest that CUSA can be safely used within defined operational parameters, provided that energy settings and exposure times are carefully managed to mitigate excessive thermal accumulation. These conclusions contribute to the understanding of the thermo-mechanical interactions in ultrasonic tumour resection and aim to assist medical professionals in optimising surgical protocols. Full article

Background: Intraoperative neurophysiological monitoring (IONM) is increasingly used during spinal surgery to reduce the risk of neurological complications. This systematic review evaluates both the clinical outcomes and cost-effectiveness of IONM in contemporary spinal surgery. Methods: A comprehensive literature search was conducted to identify studies evaluating IONM in spinal surgery. Twenty-three studies were included: twenty-one reporting clinical outcomes and two focusing on economic analysis. Data on neurological deficits, monitoring accuracy, and cost-effectiveness were extracted and analyzed. Results: Analysis of the included studies showed that IONM reduced the risk of neurological deficits across various types of spinal surgery. The diagnostic accuracy varied by modality, with MEP showing the highest sensitivity (90.2%) and SSEP demonstrating high specificity (97.1%). The greatest benefit was observed in deformity surgery and spinal tumors. D-wave monitoring showed efficacy for intramedullary tumors. Economic analysis demonstrated that IONM is cost-effective when the neurological complication rate exceeds 0.3%, with potential savings of over USD 23,000 per case. Conclusions: IONM significantly improves neurological outcomes in spinal surgery and is cost-effective in most clinical scenarios, particularly in high-risk procedures. Multimodal monitoring approaches provide the most comprehensive neurological assessment. These findings support the routine use of IONM in contemporary spinal surgery, especially for complex cases. Full article

Purpose: Intramedullary spinal cord tumors (IMSCTs) account for 2–8% of all primary CNS tumors, with ependymal tumors astrocytic tumors and hemangioblastoma being the most prevalent. Due to scarcity of large-scale studies, we aim to provide insights into the long-term neurological and functional outcomes following their resection. Methods: A single-center study where retrospective review of all patients’ medical records with IMSCT resection between October 2001 and March 2023 was conducted. Data on demographic characteristics, clinical presentations, and surgical outcomes were collected and analyzed. Results: This study included 253 patients (57.7% male) with a mean age of 36.2 ± 19. The cohort comprised ependymal tumors (45.1%), astrocytic tumors (35.6%), hemangioblastoma (11.1%), and miscellaneous tumors (n = 21; 8.3%). Differences were observed in age at surgery (p < 0.001) and mortality (p = 0.002) across tumor types. Gross total resection was more frequently achieved in hemangioblastoma (96.4%) and ependymal tumors (82.5%) compared to astrocytic tumors (55.6%) (p < 0.001). Long-term postoperative improvements were significant, with reductions in numbness from 74.7% to 52.2%, pain from 42.2% to 25.7%, and bladder incontinence from 23.7% to 11.6%, particularly in ependymal tumors and astrocytic tumors. Kaplan–Meier analysis showed that patients with ependymal tumors had the highest overall survival rates (94.8% at 5 years, 86.7% at 10 years, 76.3% at 15 years, and 65.4% at 20 years) compared to hemangioblastoma (88.7% at 5 and 10 years, and 53.2% at 15 years) and astrocytic tumors (67.8% at 5 years, 58.1% at 10 and 15 years) (p = 0.001). Conclusions: This study highlights the differences in survival and long-term functional outcomes among patients with IMSCTs based on tumor histology and grade. Full article

Introduction: Intradural extramedullary and intramedullary spinal tumors are rare, complex to treat, and require advanced surgical techniques. Ultrasonic aspirators, commonly used for tumor removal, can cause sensory and motor deficits, including loss of motor evoked potentials (MEPs). This study aims to evaluate the safety and efficacy of ultrasonic aspirators in intramedullary tumor surgery using a swine model, comparing different systems and techniques. Methods: Ten pigs underwent D1-D3 laminectomy and myelotomy, with adipose tissue simulating a tumor. The ultrasonic aspirators were tested under varying conditions (fragmentation power, suction, application time, and vibration mode). The primary endpoint is to evaluate the impact of the chosen variables on motor function damage. The secondary endpoints are histological evaluation of the type of damage caused by ultrasound aspirators and the effect of steroid drugs on MEPs’ impairment recovery. Results: Ultrasound aspirators can cause a significant MEP signal reduction when used in continuous mode, with fragmentation power >30 for more than 2 min (p < 0.001). Suction does not affect MEPs. When used in alternating/pulsatile mode, fragmentation power and application time do not affect MEPs. The two-way ANOVA analysis on the interaction between fragmentation power and application time in continuous mode did not demonstrate a significant interaction (p = 0.155). Time alone does not affect motor damage (p = 0.873). Betamethasone can restore MEPs’ signal after damage if administered immediately. Conclusions: Using ultrasonic aspirators in an animal model of intramedullary tumor surgery is safe. The main factor that resulted in the responsibility of motor function impairment is the fragmentation power. Full article

Introduction: Hemangioblastomas (HBs) are benign, highly vascular tumors that can be found intracranially or in the spinal region, representing around 2–15% of primary intramedullary tumors. They can occur sporadically or in association with Von Hipple–Lindau (VHL) disease. Despite recent of advancement of nonsurgical treatments, complete surgical resection remains the gold standard of care for the spinal HBs. Materials and Methods: We conducted an international multicenter retrospective analysis of adult patients surgically treated for spinal HBs in four European referral centers between January 2000 and September 2024, with a minimum post-operative follow-up duration of 6 months. Patients’ sex and age at surgical intervention, clinical presentation, and duration symptoms prior to clinical diagnosis were identified. The pre- and post-operative neurological status at 1 and 6 months and at the last visit was assessed using the modified McCormick score (MCS). The extent of surgical resection was divided into gross total resection (GTR) and subtotal resection (STR). Finally, post-operative complications were inspected as well, namely cerebrospinal fluid leaks, infections, hemorrhages and post-operative spinal stability. Results: A total of 35 patients were included in the cohort, with an age median of 52 years (34.5–60) and a slight male predominance (21/35, i.e., 60%). The median follow-up period was 37.5 months (12–75). More than half were located in the cervical region, making it the most common (54.3%). Syrinxes were observed in 23 cases (72%), and HBs were more commonly intramedullary (80%). GTR was achievable in around 88% of cases. Post-operative complications were observed in nine patients (25.7%). Nearly half of patients were discharged into rehabilitations centers (48.5%). Tumor recurrence was seen in 10.3% only. At the last follow-up, an excellent overall post-operative neurological status (positive ∆ McCormick) was observed in most of patients (88%) and was found to be associated with a relatively younger age group. Tumor location and presence of syrinxes did not show any statistical significance regarding clinical outcome. In patients having benefited from intra-operative monitoring, only D-wave changes showed statistical significance regarding post-operative outcome (p < 0.05). Conclusions: A large majority of patients operated for a spinal HB demonstrated favorable outcome after surgery, with unchanged or improved neurological status. Advanced age could have an impact on the post-operative neurological outcome. Other factors such as tumor size, location, and the presence of syrinx did not seem to significantly impact the neurological outcome. Finally, the surgery of these vascular lesions with no possibility of debulking or piece-meal removal and requiring “en bloc” resection is technically demanding and should be performed by experienced teams in spine and spinal cord surgery only. Full article

Osteosarcoma is medically defined as a bone-forming tumor with associated bone-degrading activity. There is a lack of knowledge about the network that generates the overproduction of bone. We studied the early stage of osteosarcoma development with mice enduring a periosteum injection of osteosarcoma cells at the proximal third of the tibia. On day 7 (D7), tumor cells activate the over-synthesis of bone-like material inside the medulla. This overproduction of bone is quickly (D13) followed by degradation. Samples were characterized by microfocus small-angle X-ray scattering (SAXS), wide-angle X-ray scattering (WAXS), optical and electron microscopies, and micro-indentation. This intramedullary apatite–collagen composite synthesis highlights an unknown network of bone synthesis stimulation by extramedullary osteosarcoma cells. This synthesis activation mechanism, coupled with the well-known bone induced osteosarcoma growth activation, produces a rare synergy that may enlighten the final osteosarcoma morphology. With this aim, a 3D cellular automaton was developed that only included two rules. Simulations can accurately reproduce the bi-continuous sponge macroscopic structure that was analyzed from mice tumor micro-tomography. This unknown tumor activation pathway of bone synthesis, combined with the known bone activation of tumor growth, generates a positive feedback synergy explaining the unusual sponge-like morphology of this bone cancer. From a biomaterials point of view, how nature controls self-assembly processes remains an open question. Here, we show how the synergy between two biological growth processes is responsible for the complex morphology of a bone tumor. This highlights how hierarchical morphologies, accurately defined from the nanometer to the centimeter scale, can be controlled by positive feedback between the self-assembly of a scaffold and the deposition of solid material. Full article

Bone metastases occur when cancer cells from the primary tumor spread to the bones. The incidence of bone metastases is increasing due to the longer survival of patients with primary tumors, driven by advances in cancer treatments. In patients with multiple bone metastases, care is primarily palliative, aiming to improve their quality of life through pain relief. Bone metastases are strongly associated with pathological fractures, particularly in the femur. In these cases, minimally invasive treatments such as percutaneous cementoplasty and internal fixation with intramedullary nails are growing in popularity. Methods: This manuscript focuses on studying these two therapies by developing virtual models using ANSYS^® software. Thermal and thermomechanical analyses were conducted to evaluate the heat effect resulting from the polymerization of different types of bone cement and to assess the benefits of combining it with internal fixation using intramedullary nails made of different materials. Results: The results highlight the advantages of combining these two techniques compared to cementoplasty alone. Furthermore, the use of Gentamicin Bone Cement (CMW 3^®) with an intramedullary nail made of either material has been shown to provide a more significant functional improvement. Conclusions: The combination of cementoplasty with internal fixation is more effective than cementoplasty alone. The use of CMW 3^® cement with an intramedullary nail made of either material provides greater control over the growth of the metastatic lesion. The chosen injection angle results in an excessive volume of cement, causing a high degree of thermal necrosis. Full article

Background/Objectives: While most studies on the postoperative condition of patients with spinal cord tumors describe long-term outcomes, data are needed on immediate surgical outcomes demanding rehabilitation to make informed assessments for postoperative planning. The aim of this study was to identify factors predicting function and rehabilitative needs after intradural spinal tumor surgery. Methods: Eighty-five prospectively recruited patients underwent surgery for intradural intramedullary (ID-IM; n = 23) and extramedullary (ID-EM; n = 62) tumors. Neurological and functional status were assessed before surgery, after surgery, and at discharge using the modified McCormick scale (MMS), Karnofsky performance status (KPS) scale, Barthel index (BI), and the gait index (GI). Results: There were no significant predictors of early postoperative rehabilitation in the ID-IM group. In the ID-EM group, age, thoracic level, subtotal resection (STR), repeat surgery, and functional scale scores predicted the need for rehabilitation. In multivariable analysis, MMS (odds ratio (OR) 8.7; 95% confidence interval (CI): 2.37–32.44) and STR (OR 13.00; 95%CI: 1.56–107.87) remained independent predictors of rehabilitation need (area under curve, 92%). Despite their younger age, most patients with ID-IM tumors, especially ependymomas, required rehabilitation but improved quickly (KPS, BI, p < 0.001). Among ID-EM tumors, meningiomas were characterized by poorer preoperative function and low gross total resection (GTR) rates, but did not deteriorate neurologically after surgery. Patients with schwannoma and ID-EM ependymomas achieved the highest GTR rate and had the best function both before and after surgery. Conclusions: These results may be useful for estimating early rehabilitation needs after intradural tumor surgery and counseling patients before surgery about the expected postoperative course. Full article

Background/Objectives: Developing high-performance artificial intelligence (AI) models for rare diseases is challenging owing to limited data availability. This study aimed to evaluate whether a novel three-class annotation method for preparing training data could enhance AI model performance in detecting osteosarcoma on plain radiographs compared to conventional single-class annotation. Methods: We developed two annotation methods for the same dataset of 468 osteosarcoma X-rays and 378 normal radiographs: a conventional single-class annotation (1C model) and a novel three-class annotation method (3C model) that separately labeled intramedullary, cortical, and extramedullary tumor components. Both models used identical U-Net-based architectures, differing only in their annotation approaches. Performance was evaluated using an independent validation dataset. Results: Although both models achieved high diagnostic accuracy (AUC: 0.99 vs. 0.98), the 3C model demonstrated superior operational characteristics. At a standardized cutoff value of 0.2, the 3C model maintained balanced performance (sensitivity: 93.28%, specificity: 92.21%), whereas the 1C model showed compromised specificity (83.58%) despite high sensitivity (98.88%). Notably, at the 25th percentile threshold, both models showed identical false-negative rates despite significantly different cutoff values (3C: 0.661 vs. 1C: 0.985), indicating the ability of the 3C model to maintain diagnostic accuracy at substantially lower thresholds. Conclusions: This study demonstrated that anatomically informed three-class annotation can enhance AI model performance for rare disease detection without requiring additional training data. The improved stability at lower thresholds suggests that thoughtful annotation strategies can optimize the AI model training, particularly in contexts where training data are limited. Full article

Objective: Due to the absence of studies supporting the role of intraoperative neurophysiological monitoring (IONM) in intradural spinal tumors, this study evaluates the clinical outcome after these surgeries in relation to the use of the advanced intraoperative neurophysiological techniques. Methods: This is an observational, descriptive and retrospective study of two cohort groups in relation to the presence or absence of IONM during the intervention and the subsequent evaluation of the clinical and functional results in the short and medium terms. Ninety-six patients with extra- or intramedullary intradural spinal tumors operated on by the neurosurgery team of our center completed the current study. Results: We observed improvements in the Prolo, Brice and McKissock and McCormick scales scores in the monitored patients. These results examine the usefulness of IONM to preserve neurological functions and, therefore, its impact on quality of life. The rate of neurological deficits in the unmonitored patients was 14.5%, whereas it was 8.3% of the patients whose treatment included IONM. Conclusions: It is important to emphasize the importance of implementing IONM for early recognition of possible neurological damage, the improvement of postoperative functional outcomes, and for decreasing the rate of neurological complications. Significance: This study provides reliable results on the importance of IONM in intradural spinal tumor surgeries. Full article

Background/Objectives: Spinal astrocytomas (SA) represent 30–40% of all intramedullary spinal cord tumors (IMSCTs) and present significant clinical challenges due to their aggressive behavior and potential for recurrence. We aimed to pool the evidence on SA and investigate predictors of regrowth or recurrence after surgical resection. Methods: A systematic review and meta-analysis were conducted on peer-reviewed human studies from several databases covering the field of SA. Data were collected including sex, age, tumor location, extent of resection, histopathological diagnosis, and adjuvant therapy to identify predictors of SA recurrence. Recurrence was defined as failure of local tumor control or regrowth after treatment. Results: A total of 53 studies with 1365 patients were included in the meta-analysis. A postoperative deterioration in neurological outcomes, as assessed by the modified McCormick scale, was noted in most of the patients. The overall recurrence rate amounted to 41%. On meta-analysis, high-grade WHO tumors were associated with higher odds of recurrence (OR = 2.65; 95% CI: 1.87, 3.76; p = 0.001). Similarly, GTR was associated with lower odds of recurrence compared to STR (OR = 0.33; 95% CI: 0.18, 0.60; p = 0.0003). Sex (p = 0.5848) and tumor location (p = 0.3693) did not show any significant differences in the odds of recurrence. Intraoperative neurophysiological monitoring was described in 8 studies and adjuvant radiotherapy in 41 studies. Conclusions: The results highlight the significant importance of tumor grade and extent of resection in patient prognosis. The role of adjuvant radiotherapy remains unclear, with most studies suggesting no differences in outcomes, with limitations due to potential confounders. Full article

Intramedullary spinal cord glioblastoma (ISCG) is lethal due to lack of effective treatment. We previously established a rat C6-ISCG model and the antitumor effect of F3.CD-TK, an hNSC line expressing CD and TK, via producing cytocidal 5FU and GCV-TP. However, the neurotherapeutic potential of this hNSC approach has remained uninvestigated. Here for the first time, cultured F3.CD-TK cells were found to have a markedly higher oncolytic effect, which was GJIC-dependent, and BDNF expression but less VEGF secretion than F3.CD. In Rowett athymic rats, F3.CD-TK (1.5 × 10⁶ cells/10 µL × 2), injected near C6-ISCG (G55 seeding 7 days earlier: 10 K/each) and followed by q.d. (×5/each repeat; i.p.) of 5FC (500 mg/kg/5 mL/day) and GCV (25 mg/kg/1 mL/day), robustly mitigated cardiorespiratory, locomotor, and sensory deficits to improve neurofunction and overall survival compared to animals receiving either F3.CD or F3.CD-TK+F3.CD debris formula. The F3.CD-TK regimen exerted greater tumor penetration and neural inflammation/immune modulation, reshaped C6-ISCG topology to increase the tumor’s surface area/volume ratio to spare/repair host axons (e.g., vGlut1+ neurites), and had higher post-prodrug donor self-clearance. The multimodal data and mechanistic leads from this proof-of-principle study suggest that the overall stronger anti-ISCG benefit of our hNSC-based GDEPT is derived from its concurrent oncolytic and neurotherapeutic effects. Full article