Search Results (16)

Background/Objectives: Spinal ependymoma is the most common intramedullary spinal cord tumor in adults, and maximal safe resection is the cornerstone of treatment. Patients aged 75 years and older are underrepresented in surgical neuro-oncology cohorts. We sought to characterize treatment patterns and identify predictors of overall survival in very elderly patients with spinal ependymoma. Methods: We performed a retrospective cohort study of patients aged 65 years or older with spinal ependymoma using the National Cancer Database. The primary cohort was patients aged 75 years or older (very elderly); patients aged 65–74 years served as a comparison cohort. Multivariable Cox proportional-hazards models were fit within each cohort, and a surgery-by-age-cohort interaction was tested. Results: Of 1497 eligible patients aged 65 years or older with spinal ependymoma, 422 patients (28.2%) met criteria for the final analytic cohort. Intramedullary versus extramedullary tumor status was not available in the NCDB PUF and therefore could not be characterized. Very elderly patients were less likely to undergo surgery than the comparison cohort (70% vs. 85%; p < 0.001) despite similar tumor characteristics. Among very elderly patients, median overall survival was 59.7 months without surgery and 106.0 months with surgery, an approximately 46-month difference favoring surgery. Surgery was independently associated with lower mortality (HR 0.46; 95% CI, 0.24–0.89; p = 0.021). Increasing age (HR 1.15 per year; 95% CI, 1.07–1.22; p < 0.001), Charlson–Deyo score ≥ 2 (HR 4.41; 95% CI, 1.65–11.79; p = 0.003), and increasing tumor size (HR 1.02 per mm; 95% CI, 1.01–1.04; p < 0.001) were also independently associated with worse survival. In the 65–74 cohort, no significant association between surgery and overall survival was detected (HR 1.23; 95% CI, 0.54–2.81; p = 0.623), though statistical power was limited by only 7 deaths in the no-surgery arm. The surgery-by-age-cohort interaction was significant (HR 0.37; p = 0.043). Conclusions: Surgical resection was independently associated with improved overall survival in very elderly patients with spinal ependymoma despite lower utilization. Chronological age alone may be an imperfect basis for excluding older adults from surgical consideration. Full article

Background/Objectives: Intramedullary tumors are uncommon spinal cord lesions that account for a small proportion of central nervous system neoplasms but are associated with a high risk of neurological morbidity. Accurate preoperative characterization is essential because therapeutic strategies, surgical planning, and functional prognosis depend strongly on tumor biology and growth behavior within the confined spinal cord environment. This study aims to characterize the radiological phenotype of intramedullary tumors and to identify imaging patterns that may assist in lesion characterization and diagnostic stratification. Methods: A retrospective analysis of preoperative MRI findings in patients with histopathologically confirmed intramedullary tumors was performed. Preoperative MRI examinations were systematically analyzed to describe imaging features according to tumor histology using conventional sequences (T1-weighted, T2-weighted, and contrast-enhanced imaging). Results: Distinct radiological phenotypes were observed across a wide spectrum of lesions. Glial tumors, including subependymoma, ependymoma, pilocytic astrocytoma, diffuse midline glioma H3K27M, glioblastoma, high-grade astrocytoma with piloid features, ganglioglioma, and diffuse leptomeningeal glioneural tumors, demonstrated variable combinations of cord expansion, margin definition, enhancement patterns, and tract involvement, reflecting differences between expansile and infiltrative growth. Secondary tumors such as metastases frequently exhibited aggressive imaging features, including extensive edema and intense or heterogeneous enhancement. Vascular lesions, including hemangioblastoma and cavernoma, showed characteristic vascular signatures, such as nodular enhancement with flow voids or susceptibility-related signal changes. Developmental lesions, such as epidermoid cysts, neurenteric cysts, and lipoma, displayed distinctive signal characteristics, especially on diffusion and T1, that aided differentiation from neoplastic processes. Conclusions: In conclusion, the structured radiological interpretation functions proposed herein are not only useful for diagnostic purposes, but could also be useful for risk stratification and therapeutic guidance. Full article

Spinal tumours are an uncommon but significant cause of pain, fractures, instability, and cord compression, leading to poor quality of life and mortality. Separation surgery is a rapidly advancing technique that has seen increased utilisation in the field of spinal oncology surgery. Separation surgery can be described as a resection technique that decompresses the spinal cord whilst creating an ablative target for high-dose stereotactic radiotherapy to achieve durable local control while minimising the risk of radiation myelopathy. This has facilitated the delivery of stereotactic radiotherapy, as well as created potential for use in managing primary bone tumours of the spine. From a radiology standpoint, optimal outcomes depend on meticulous preoperative characterisation of tumour volume and stability (e.g., ESCC grade and SINS), clear communication of anatomic constraints relevant to approach and fixation, and systematic postoperative surveillance to distinguish expected postoperative appearances from early recurrence or complications. We present our radiological experience and report recommendations while evaluating spinal oncology separation surgery. Full article

Human hemangioblastoma is a benign, slow-growing, highly vascular neoplasm. The tumor most commonly arises in the cerebral hemispheres and cerebellum, where it is more frequently observed in patients with von Hippel–Lindau disease. In veterinary medicine, hemangioblastoma has only been described in the central nervous system of dogs and in the skin of lambs. Our study aimed to characterize the clinical and neuropathological features of five cases of canine spinal cord hemangioblastoma and one case of sciatic nerve localization, and to compare these results with those reported in the veterinary literature. Diagnoses were achieved by neurological examination, neuroimaging, surgery or post-mortem examination, histopathology, and immunohistochemistry. All tumors were composed of numerous, haphazardly arranged capillaries lined by plump endothelium and interstitial fusiform to stellate stromal cells. Immunohistochemically, the stromal cells were strongly immunolabeled with NSE and carbonic anhydrase IX and were negative for von Willebrand factor VIII and inhibin-α. Canine hemangioblastoma exhibits morphological and immunohistochemical features comparable to the human counterpart, although the latter is mostly positive for inhibin-α. Surgery may be effective in cases of intradural-extramedullary and peripheral nerve locations, as in humans. This is the first report of peripheral nerve hemangioblastoma in animals. Full article

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: Spinal solitary fibrous tumors (SFTs) are a rare oncological entity, almost anecdotal in the pediatric population. They have a high relapse rate and represent an ongoing oncological challenge. Methods: In this article, we conducted a systematic review starting from a case report to highlight the current state of the art in managing these tumors. Results: Spinal solitary fibrous tumors (SFTs) are rare, slow-growing neoplasms that can be either intra- or extramedullary. Only a limited number of studies focus on primary pediatric spinal cord localization. Five pediatric cases of spinal SFT have been documented in the literature. On MRI, they typically present as highly vascularized, contrast-enhancing masses. Histologically, they are composed of spindle-shaped cells within a collagenous stroma featuring staghorn-shaped blood vessels. More aggressive subtypes, such as dedifferentiated SFTs, resemble high-grade sarcomas. The NAB2–STAT6 fusion is a key marker, driving EGFR signaling, collagen production, and fibrosis. Additional diagnostic markers include CD34, CD99, and Bcl-2. Surgical resection remains the primary treatment. In metastatic cases, chemotherapy—mainly with anthracyclines, dacarbazine, or temozolomide—is employed, although no standardized pediatric protocols exist. Anti-angiogenic agents, including tyrosine kinase inhibitors, have shown promise. Radiotherapy is used postoperatively for local disease control, but its impact on survival is still under investigation. Conclusions: Surgery remains the cornerstone of treatment, significantly impacting the natural history of the disease and symptom control. While clinical trials exploring radiotherapy and chemotherapy are ongoing in adults, no specific treatment protocol has been established for pediatric patients. Full article

Background: Ependymomas are primary CNS neoplasms that arise from the ependymal cells of the brain and spinal cord, accounting for 3–6% of all CNS tumors. Aims: This study provides a comprehensive analysis of ependymoma survival patterns and examines non-cancer causes of death in the US. Methods: This retrospective study used data from SEER 17 registries between 2000 and 2019 to evaluate the incidence of ependymoma, as well as the survival and mortality trends in the US. Results: A total of 3821 patients were included, with 842 (22%) deaths. The highest mortality was observed in younger patients (<18 years) within one year of diagnosis (SMR, 54.77; 95% CI, 38.95–74.88). Brain and other nervous system cancers were the leading causes of death, followed by non-cancer causes, particularly cerebrovascular diseases, pneumonia, influenza, and septicemia. The survival rates observed at one, three, and five years were 94% (95% CI: 0.94–0.95), 88% (95% CI: 0.87–0.89), and 84% (95% CI: 0.82–0.85), respectively. Conditional survival improved over time, with a three-year conditional relative survival rate of 92% after one year of diagnosis and 96% for those who survived five years. Conclusion: The death rate was highest among pediatric patients under 18 years of age. Cerebrovascular disorders were the leading non-cancer cause of death across all time intervals. The probability of surviving for three years increases for patients who have already survived one, three, or five years post-diagnosis. Full article

Myeloproliferative neoplasm (MPN) with eosinophilia associated with FIP1L1-PDGFRA is a rare eosinophilic disorder typically treated with imatinib. However, resistance due to the T674I mutation poses a significant challenge. This case presents the first reported instance of concurrent FIP1L1-PDGFRA T674I and PTPN11 (p.E76D) mutations in a 38-year-old male patient with MPN and eosinophilia. The patient initially responded to imatinib but developed resistance after ten months, leading to severe spinal cord compression caused by granulocytic sarcoma. Despite undergoing radiotherapy, chemotherapy, and allogeneic hematopoietic stem cell transplantation (allo-HSCT), the disease progressed. Although full donor chimerism was achieved post-transplant, the patient relapsed shortly afterward with eosinophilia, splenomegaly, and constitutional symptoms. Further treatments, including sorafenib and decitabine, failed to control the disease, and the patient ultimately died from multiorgan failure. This case illustrates the therapeutic challenges associated with FIP1L1-PDGFRA T674I-positive eosinophilic disorder, especially when compounded by the PTPN11 mutation. Resistance to standard treatments underscores the urgent need for novel therapies to manage this rare and aggressive disease. Full article

Radiation therapy is widely recognized as an efficacious modality for treating neoplasms located within the craniofacial region. Nevertheless, this approach is not devoid of risks, predominantly concerning potential harm to the neural structures. Adverse effects may encompass focal cerebral necrosis, cognitive function compromise, cerebrovascular pathology, spinal cord injury, and detriment to the neural fibers constituting the brachial plexus. With increasing survival rates among oncology patients, evaluating post-treatment quality of life has become crucial in assessing the benefits of radiation therapy. Consequently, it is imperative to investigate therapeutic strategies to mitigate cerebral complications from radiation exposure. Current management of radiation-induced cerebral damage involves corticosteroids and bevacizumab, with preclinical research on antioxidants and thalidomide. Despite these efforts, an optimal treatment remains elusive. Recent studies suggest the gut microbiota’s involvement in neurologic pathologies. This review aims to discuss the causes and existing treatments for radiation-induced cerebral injury and explore gut microbiota modulation as a potential therapeutic strategy. Full article

Differentiating neoplastic from non-neoplastic spinal cord pathologies may be challenging due to overlapping clinical and radiological features. Spinal cord tumors, which comprise only 2–4% of central nervous system tumors, are rarer than non-tumoral myelopathies of inflammatory, vascular, or infectious origins. The risk of neurological deterioration and the high rate of false negatives or misdiagnoses associated with spinal cord biopsies require a cautious approach. Facing a spinal cord lesion, prioritizing more common non-surgical myelopathies in differential diagnoses is essential. A comprehensive radiological diagnostic approach is mandatory to identify spinal cord tumor mimics. The diagnostic process involves a multi-step approach: detecting lesions primarily using MRI techniques, precise localization of lesions, assessing lesion signal intensity characteristics, and searching for potentially associated anomalies at spinal cord and cerebral MRI. This review aims to delineate the radiological diagnostic approach for spinal cord lesions that may mimic tumors and briefly highlight the primary pathologies behind these lesions. Full article

Spinal cord tumors are infrequently identified spinal diseases that are often difficult to diagnose even with magnetic resonance imaging (MRI) findings. To minimize the probability of overlooking these tumors and improve diagnostic accuracy, an automatic diagnostic system is needed. We aimed to develop an automated system for detecting and diagnosing spinal schwannomas and meningiomas based on deep learning using You Only Look Once (YOLO) version 4 and MRI. In this retrospective diagnostic accuracy study, the data of 50 patients with spinal schwannomas, 45 patients with meningiomas, and 100 control cases were reviewed, respectively. Sagittal T1-weighted (T1W) and T2-weighted (T2W) images were used for object detection, classification, training, and validation. The object detection and diagnosis system was developed using YOLO version 4. The accuracies of the proposed object detections based on T1W, T2W, and T1W + T2W images were 84.8%, 90.3%, and 93.8%, respectively. The accuracies of the object detection for two spine surgeons were 88.9% and 90.1%, respectively. The accuracies of the proposed diagnoses based on T1W, T2W, and T1W + T2W images were 76.4%, 83.3%, and 84.1%, respectively. The accuracies of the diagnosis for two spine surgeons were 77.4% and 76.1%, respectively. We demonstrated an accurate, automated detection and diagnosis of spinal schwannomas and meningiomas using the developed deep learning-based method based on MRI. This system could be valuable in supporting radiological diagnosis of spinal schwannomas and meningioma, with a potential of reducing the radiologist’s overall workload. Full article

Primary brain and central nervous system (CNS) tumors are a diverse group of neoplasms that occur within the brain and spinal cord. Although significant advances in our understanding of the intricate biological underpinnings of CNS neoplasm tumorigenesis and progression have been made, the translation of these discoveries into effective therapies has been stymied by the unique challenges presented by these tumors’ exquisitely sensitive location and the body’s own defense mechanisms (e.g., the brain–CSF barrier and blood–brain barrier), which normally protect the CNS from toxic insult. These barriers effectively prevent the delivery of therapeutics to the site of disease. To overcome these obstacles, new methods for therapeutic delivery are being developed, with one such approach being the utilization of nanoparticles. Here, we will cover the current state of the field with a particular focus on the challenges posed by the BBB, the different nanoparticle classes which are under development for targeted CNS tumor therapeutics delivery, and strategies which have been developed to bypass the BBB and enable effective therapeutics delivery to the site of disease. Full article

Spinal cord tumors constitute a diverse group of rare neoplasms associated with significant mortality and morbidity that pose unique clinical and surgical challenges. Diagnostic accuracy and outcome prediction are critical for informed decision making and can promote personalized medicine and facilitate optimal patient management. Machine learning has the ability to analyze and combine vast amounts of data, allowing the identification of patterns and the establishment of clinical associations, which can ultimately enhance patient care. Although artificial intelligence techniques have been explored in other areas of spine surgery, such as spinal deformity surgery, precise machine learning models for spinal tumors are lagging behind. Current applications of machine learning in spinal cord tumors include algorithms that improve diagnostic precision by predicting genetic, molecular, and histopathological profiles. Furthermore, artificial intelligence-based systems can assist surgeons with preoperative planning and surgical resection, potentially reducing the risk of recurrence and consequently improving clinical outcomes. Machine learning algorithms promote personalized medicine by enabling prognostication and risk stratification based on accurate predictions of treatment response, survival, and postoperative complications. Despite their promising potential, machine learning models require extensive validation processes and quality assessments to ensure safe and effective translation to clinical practice. Full article

Multiple myeloma (MM) is a B-cell neoplasm characterized by clonal plasma–cell proliferation. The survival and prognosis of this condition have been significantly improved by treatment with active anti-MM drugs such as bortezomib or lenalidomide. Further, the discovery of novel agents has recently paved the way for new areas of investigation. However, MM, including myeloma-related bone diseases, remains fatal. Bone disease or bone destruction in MM is a consequence of skeletal involvement with bone pain, spinal cord compression, and bone fracture resulting from osteolytic lesions. These consequences affect disease outcomes, including patients’ quality of life and survival. Several studies have sought to better understand MM bone disease (MBD) through the classification of its molecular mechanisms, including osteoclast activation and osteoblast inhibition. Bisphosphonates and the receptor activator of the nuclear factor-kappa B (NF-κB) ligand (RANKL) inhibitor, denosumab, prevent skeletal-related events in MM. In addition, several other bone-targeting agents, including bone-anabolic drugs, are currently used in preclinical and early clinical evaluations. This review summarizes the current knowledge of the pathogenesis of MBD and discusses novel agents that appear very promising and will soon enter clinical development. Full article