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Background: Primary malignant tumors of the spine are rare and most commonly occur in lumbar and thoracic vertebrae. We report a rare case of retroperitoneal chondrosarcoma of L3 that was managed with sagittal en bloc spondylectomy following chemoradiation., Case Description: A 26-year-old woman was evaluated for abdominal pain with contrast-enhanced computed tomography of the abdomen and pelvis, which revealed a soft tissue retroperitoneal mass arising from L3. She underwent laparotomy and biopsy, which revealed chondrosarcoma, and she received chemoradiation over a period of 28 weeks 6 days. After repeat imaging, she underwent single-stage combined approach sagittal en bloc spondylectomy of retroperitoneal chondrosarcoma of L3 with right nephrectomy and spine reconstruction. At 3-year follow-up, there was no evidence of recurrence on contrast-enhanced computed tomography of the abdomen and pelvis. She demonstrated no gait abnormality or spinal deformity., Conclusions: Sagittal en bloc spondylectomy is a preferred surgical approach for eccentrically placed spinal tumors that offers better oncological and functional outcomes., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: We aim to construct a practical clinical prediction model to accurately evaluate the overall survival (OS) of patients with primary spinal tumors after primary tumor resection, thereby aiding clinical decision-making., Methods: A total of 695 patients diagnosed with a primary spinal tumor, selected from the Surveillance, Epidemiology, and End Results (SEER) database, were included in this study. The Cox regression algorithm was applied to the training cohort to build the prognostic nomogram model. The nomogram's performance in terms of discrimination, calibration, and clinical usefulness was also assessed in the internal SEER validation cohort. The fitted prognostic nomogram was then used to create a web-based calculator., Results: Four independent prognostic factors were identified to establish a nomogram model for patients with primary spinal tumors who had undergone surgical resection. The C-index (.757 for the training cohort and .681 for the validation cohort) and the area under the curve values over time (both >.68) showed that the model exhibited satisfactory discrimination in both the SEER cohort. The calibration curve revealed that the projected and actual survival rates are very similar. The decision curve analysis also revealed that the model is clinically valuable and capable of identifying high-risk patients., Conclusions: After developing a nomogram and a web-based calculator, we were able to reliably forecast the postoperative OS of patients with primary spinal tumors. These tools are expected to play an important role in clinical practice, informing clinicians in making decisions about patient care after surgery.

Background: Mesenchymal chondrosarcoma (MCS) is an aggressive subtype of chondrosarcoma that occurs extremely rarely in the central nervous system. Patients often present with pain or sensorimotor deficits, and resection is considered the gold standard. The role of adjuvant radiation and/or chemotherapy is largely unknown., Observations: A 22-year-old male presented with a 4-month history of progressive back and bilateral leg pain. He underwent imaging workup with magnetic resonance imaging of the lumbar spine and was found to have an intradural, extramedullary, heterogeneously enhancing mass spanning the L4-5 vertebral levels. Intraoperatively, a lobular, partially calcified mass with a ventral dural attachment displacing the nerve roots laterally was observed. The mass was removed en bloc, and the patient later underwent adjuvant radiotherapy, with no evidence of recurrence 2 years following surgery., Lessons: Spinal MCS is extremely rare and often presents with a more aggressive course than conventional chondrosarcoma. Radiological diagnosis is challenging, as the tumor mimics different pathologies. The presence of calcifications, heterogeneous enhancement, and a more rapid clinical course as well as the presence of HEY1::NCOA2 gene fusion, which can be detected by surrogate immunohistochemistry, aids in diagnosis. Resection is the standard of care, and adjuvant radiation may be considered to reduce local recurrence, although further studies are warranted.

Spine tumors represent a significant social and medical problem, affecting the quality of life of thousands of patients and imposing a burden on healthcare systems worldwide. Encompassing a wide range of diseases, spine tumors require prompt multidisciplinary treatment strategies, being mainly approached through chemotherapy, radiotherapy, and surgical interventions, either alone or in various combinations. However, these conventional tactics exhibit a series of drawbacks (e.g., multidrug resistance, tumor recurrence, systemic adverse effects, invasiveness, formation of large bone defects) which limit their application and efficacy. Therefore, recent research focused on finding better treatment alternatives by utilizing modern technologies to overcome the challenges associated with conventional treatments. In this context, the present paper aims to describe the types of spine tumors and the most common current treatment alternatives, further detailing the recent developments in anticancer nanoformulations, personalized implants, and enhanced surgical techniques.

Background Context: Primary malignant non-osseous spinal tumors are relatively rare and this has led to a paucity of studies specifically examining the epidemiology of malignant spinal tumors., Purpose: To provide an updated and more comprehensive study examining the epidemiology and relative survival of these rare tumors., Study Design/setting: Data was retrospectively acquired from the Central Brain Tumor Registry of the United States (CBTRUS)., Patient Sample: Primary malignant non-osseous spinal tumor cases diagnosed between 2000 and 2017 in the United States., Outcome Measures: Incidence rates (IRs), relative survival rates, and hazard ratios (HR) were measured., Methods: IRs were calculated only for histologically-confirmed cases between 2000 and 2017. Relative survival estimates were calculated from survival information on malignant spinal tumors between 2001 and 2016 for death from any cause. Multivariable Cox proportional hazards regression models were constructed to control for age, sex, race, and ethnicity., Results: From 2000 to 2017, approximately 587 new cases of malignant non-osseous spinal tumors were diagnosed every year in the United States. The overall IR was 0.178 per 100,000 persons. Ependymomas were the most commonly diagnosed tumor in all age groups. The 10-year relative survival rates were 94.1%, 62.1%, 62.0%, and 13.3% for ependymomas, lymphomas, diffuse astrocytomas, and high-grade astrocytomas, respectively. Females have a significantly lower risk of death as compared with males for ependymomas (HR: 0.74, p<.001) and diffuse astrocytomas (HR: 0.70, p=.005). African-Americans have a significantly higher risk of death compared with Caucasians when diagnosed with ependymomas (HR: 1.52, p=.009) or lymphomas (HR: 1.55, p=.009)., Conclusion: Primary malignant non-osseous spinal tumors are primarily diagnosed in adulthood or late adulthood. Ependymal tumors are the most commonly diagnosed primary malignant non-osseous spinal tumors and have the highest 10-year relative survival rates. High-grade astrocytomas are rare and portend the worst prognosis., (Copyright © 2022 Elsevier Inc. All rights reserved.)

The field of spinal oncology has substantially evolved over the past decades. This review synthesizes and appraises what was learned and what will potentially be discovered from the recently completed and ongoing clinical studies related to the treatment of primary and secondary spinal neoplasms. This scoping review included all clinical studies on the treatment of spinal neoplasms registered in the ClinicalTrials.gov website from February 2000 to December 2020. The terms "spinal cord tumor," "spinal metastasis," and "metastatic spinal cord compression" were used. Of the 174 registered clinical studies on primary spinal tumors and spinal metastasis, most of the clinical studies registered in this American registry were interventional studies led by single institutions in North America (n = 101), Europe (n = 43), Asia (n = 24), or other continents (n = 6). The registered clinical studies mainly focused on treatment strategies for spinal neoplasms (90.2%) that included investigating stereotactic radiosurgery (n = 33), radiotherapy (n = 21), chemotherapy (n = 20), and surgical technique (n = 11). Of the 69 completed studies, the results from 44 studies were published in the literature. In conclusion, this review highlights the key features of the 174 clinical studies on spinal neoplasms that were registered from 2000 to 2020. Clinical trials were heavily skewed toward the metastatic population as opposed to the primary tumors which likely reflects the rarity of the latter condition and associated challenges in undertaking prospective clinical studies in this population. This review serves to emphasize the need for a focused approach to enhancing translational research in spinal neoplasms with a particular emphasis on primary tumors., (© The Author(s) 2021. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: In 2020, the World Health Organization (WHO) published the 5 th version of the soft tissue and bone tumor classification. Based on this novel classification system, we reviewed the current knowledge on all tumor entities with spinal manifestations, their biologic behavior, and most importantly the appropriate treatment options as well as surgical approaches., Methods: All tumor entities were extracted from the WHO Soft-Tissue and Bone Tumor Classification (5 th Edition). PubMed and Google Scholar were searched for the published cases of spinal tumor manifestations for each entity, and the following characteristics were extracted: Growth pattern, ability to metastasize, peak age, incidence, treatment, type of surgical resection indicated, recurrence rate, risk factors, 5-year survival rate, key molecular or genetic alterations, and possible associated tumor syndromes. Surgical treatment strategies as well as nonsurgical treatment recommendations are presented based on the biologic behavior of each lesion., Results: Out of 163 primary tumor entities of bone and soft tissue, 92 lesions have been reported along the spinal axis. Of these 92 entities, 54 have the potential to metastasize. The peak age ranges from conatal lesions to 72 years. For each tumor entity, we present recommended surgical treatment strategies based on the ability to locally destruct tissue, to grow, recur after resection, undergo malignant transformation as well as survival rates. In addition, potential systemic treatment recommendations for each tumor entity are outlined., Conclusion: Based on the 5 th Edition of the WHO bone and soft tumor classification, we identified 92 out of 163 tumor entities, which potentially can have spinal manifestations. Exact preoperative tissue diagnosis and interdisciplinary case discussions are crucial. Surgical resection is indicated in a significant subset of patients and has to be tailored to the specific biologic behavior of the targeted tumor entity based on the considerations outlined in detail in this article., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Journal of Craniovertebral Junction and Spine.)

Objective: Oncological resection of primary spine tumors is associated with lower recurrence rates. However, even in the most experienced hands, the execution of a meticulously drafted plan sometimes fails. The objectives of this study were to determine how successful surgical teams are at achieving planned surgical margins and how successful surgeons are in intraoperatively assessing tumor margins. The secondary objective was to identify factors associated with successful execution of planned resection., Methods: The Primary Tumor Research and Outcomes Network (PTRON) is a multicenter international prospective registry for the management of primary tumors of the spine. Using this registry, the authors compared 1) the planned surgical margin and 2) the intraoperative assessment of the margin by the surgeon with the postoperative assessment of the margin by the pathologist. Univariate analysis was used to assess whether factors such as histology, size, location, previous radiotherapy, and revision surgery were associated with successful execution of the planned margins., Results: Three hundred patients were included. The surgical plan was successfully achieved in 224 (74.7%) patients. The surgeon correctly assessed the intraoperative margins, as reported in the final assessment by the pathologist, in 239 (79.7%) patients. On univariate analysis, no factor had a statistically significant influence on successful achievement of planned margins., Conclusions: In high-volume cancer centers around the world, planned surgical margins can be achieved in approximately 75% of cases. The morbidity of the proposed intervention must be balanced with the expected success rate in order to optimize patient management and surgical decision-making.

Objective: Three-dimensional (3D) printing has emerged as a visualization tool for clinicians and patients. We sought to use patient-specific 3D-printed anatomic modeling for preoperative planning and live intraoperative guidance in a series of complex primary spine tumors., Methods: Over 9 months, patients referred to a single neurosurgical provider for complex primary spinal column tumors were included. Most recent spinal magnetic resonance and computed tomography (CT) imaging were semiautomatically segmented for relevant anatomy and models were printed using polyjet multicolor printing technology. Models were available to surgical teams before and during the operative procedure. Patients also viewed the models preoperatively during surgeon explanation of disease and surgical plan to aid in their understanding., Results: Tumor models were prepared for 9 patients, including 4 with chordomas, 2 with schwannomas, 1 with osteosarcoma, 1 with chondrosarcoma, and 1 with Ewing-like sarcoma. Mean age was 50.7 years (range, 15-82 years), including 6 males and 3 females. Mean tumor volume was 129.6 cm 3 (range, 3.3-250.0 cm 3 ). Lesions were located at cervical, thoracic, and sacral levels and were treated by various surgical approaches. Models were intraoperatively used as patient-specific anatomic references throughout 7 cases and were found to be technically useful by the surgical teams., Conclusions: We present the largest case series of 3D-printed spine tumor models reported to date. 3D-printed models are broadly useful for operative planning and intraoperative guidance in spinal oncology surgery., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Pediatric primary spinal tumors (PST) are fairly uncommon, with little available data regarding incidence and outcomes., Materials and Methods: We conducted a retrospective review of the 22 consecutive patients less than 18 years old diagnosed with PST between March 1997 and May 2011 and treated at Chang Gung Children Hospital. All patients had undergone magnetic resonance imaging (MRI) for pre-operative evaluation and operations for PST. The extent of tumor removal was assessed by surgical report by the neurosurgeon or by post-operative MRI if available., Results: Ten of them had intramedullary tumors and 12 had intradural extramedullary tumors. All patients were treated with surgery to the primary site. A total of 15 patients underwent gross total tumor resection and seven patients received post-operative radiotherapy. Five patients received adjuvant chemotherapy for their primary tumor. Fourteen patients (64%) survived from study entry without tumor progression., Conclusions: PST encompassed a diverse group of pathologic entities that differ markedly based on the location and age of the children. Total resection of pediatric PST in children could be performed with acceptable risk and satisfactory long-term results.

Introduction: To describe a single case, the fourth ever reported, of pleomorphic liposarcoma of the spine and to undertake a review of the literature., Presentation of Case: A 60 year old male patient had a bilateral lumbosciatica over a 3 month period. Imaging tests revealed a tumor mass in L1-L3 and a fracture in L2. Also, he had a mural thrombus both in the inferior vena cava and the left renal vein. The biopsy revealed a well-differentiated liposarcoma. En bloc resection of the lesion and stabilization was carried out. Due to the condition of the patient (hemodynamic instability, wound dehiscence and infection, and hypoproteinemia), a decision was made not to subject the patient to either radiation therapy or chemotherapy. The patient was subsequently found to suffer from myopathic paraparesis and a surgical wound infection. At three months, liver metastases were evident, as well as a recurrence of the lesion. A venous thrombosis that extended from the lower iliac vein to the right atrium was observed. The patient died from type I hepatorenal syndrome., Discussion: Pleomorphic liposarcoma of the spine is a rare occurrence. En bloc resection with wide margins is the treatment of choice. The use of radiotherapy in the spine is controversial. The role that should be played by chemotherapy is still unclear, although it has been employed in treatments., Conclusion: In spite of treatment, these tumors lead to a poor prognosis, with high rates of recurrence, metastasis, and mortality., (Copyright © 2016 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Intramedullary, intradural/extramedullary, and extradural spine tumors comprise a wide range of neoplasms with an even wider range of clinical symptoms and prognostic features. Magnetic resonance imaging (MRI), commonly used to evaluate the spine in patients presenting with pain, can further characterize lesions that may be encountered on other imaging studies, such as bone scintigraphy or computed tomography (CT). The advantage of the MRI is its multiplane capabilities, superior contrast agent resolution, and flexible protocols that play an important role in assessing tumor location, extent in directing biopsy, in planning proper therapy, and in evaluating therapeutic results. A multimodality approach can be used to fully characterize the lesion and the combination of information obtained from the different modalities usually narrows the diagnostic possibilities significantly. The diagnosis of spinal tumors is based on patient age, topographic features of the tumor, and lesion pattern, as seen at CT and MRI. The shift to high-end imaging incorporating diffusion-weighted imaging, diffusion tensor imaging, magnetic resonance spectroscopy, whole-body short tau inversion recovery, positron emission tomography, intraoperative and high-field MRI as part of the mainstream clinical imaging protocol has provided neurologists, neuro-oncologists, and neurosurgeons a window of opportunity to assess the biologic behavior of spine neoplasms. This chapter reviews neuroimaging of spine tumors, primary and secondary, discussing routine and newer modalities that can reduce the significant morbidity associated with these neoplasms., (© 2016 Elsevier B.V. All rights reserved.)

Purpose: Primary spinal cord and appendage tumors (PSCAT) originating from the spinal cord, spinal meninges, and cauda equina are uncommon. Worldwide, population-based cancer registry data are mostly based on malignant tumors only, which means few data are available on PSCATs, including non-malignant tumors. Therefore, the objective of this study was to provide information regarding the incidence of both non-malignant and malignant PSCATs in Korea on a national level., Materials and Methods: Incidence of PSCATs was estimated from cases diagnosed between 2006 and 2010 using the National Cancer Incidence Database in Korea. Age-adjusted rates were calculated using the world standard population, and male-to-female rate ratios were calculated by histology type., Results: Of all PSCATs registered (n=3,312), 86.6% were non-malignant. The overall age-adjusted incidence of PSCATs was 1.08 per 100,000 person-years, with an incidence of 0.99 per 100,000 in females and 1.15 in males. The most common site of PSCATs was the spinal cord (83.4%), followed by spinal meninges (16.1%) and cauda equina (0.5%). The most common histological type was neurilemmoma (41.3%), followed by meningiomas (20.1%) and ependymomas (7.6%). Men had significantly higher rates than women for ependymomas and lymphomas but had lower rates for meningiomas., Conclusion: This study provides the first population-based analysis of PSCATs in Korea.

Primary spinal tumors are rare diseases. Primary spinal tumor registry would be useful to help decision making in this complex field of spine surgery. In this article the authors present the latest findings from the Primary Spinal Tumor Registry at the National Centre for Spinal Disorders, Hungary. The registry is based on a novel database management software, the REDCap electronic data capture system. It contains data of 323 patients treated surgically during an 18-year period. Among the 126 malignant tumors, the most frequent was chordoma (61 cases). In the case of benign tumors schwannoma showed the largest prevalence (45 cases). The authors conclude that due to the rarity of the disease and the complexity of the management, multicenter, prospective registries are required to provide high level of evidence. The structure of the Primary Spinal Tumor Registry in the National Centre for Spinal Disorders in Hungary is optimal for user-friendly, fast and secure data collection providing a prospective database for scientific researches and clinical follow-up.