Diffusion tensor imaging in unclear intramedullary tumor-suspected lesions allows separating tumors from inflammation

Design Prospective diagnostic study. Objectives Primary imaging-based diagnosis of spinal cord tumor-suspected lesions is often challenging. The identification of the definite entity is crucial for dedicated treatment and therefore reduction of morbidity. The aim of this trial was to investigate specific quantitative signal patterns to differentiate unclear intramedullary tumor-suspected lesions based on diffusion tensor imaging (DTI). Setting Medical Center - University of Freiburg, Germany. Methods Forty patients with an unclear tumor-suspected lesion of the spinal cord prospectively underwent DTI. Primary diagnosis was determined by histological or clinical work-up or remained indeterminate with follow-up. DTI metrics (FA/ADC) were evaluated at the central lesion area, lesion margin, edema, and normal spinal cord and compared between different diagnostic groups (ependymomas, other spinal cord tumors, inflammations). Results Mean DTI metrics for all spinal cord tumors (n = 18) showed significantly reduced FA and increased ADC values compared to inflammatory lesions (n = 8) at the lesion margin (p < 0.001, p = 0.001) and reduced FA at the central lesion area (p < 0.001). There were no significant differences comparing the neoplastic subgroups of ependymomas (n = 10) and other spinal cord tumors (n = 8), but remaining differences for both compared to the inflammation subgroup. We found significant higher ADC (p = 0.040) and a trend to decreased FA (p = 0.081) for ependymomas compared to inflammations at the edema. Conclusion Even if distinct differentiation of ependymomas from other spinal cord neoplasms was not possible based on quantitative DTI metrics, FA and ADC were feasible to separate inflammatory lesions. This may avoid unnecessary surgery in patients with unclear intramedullary tumor-suspected lesions.

Analysis of Factors Influencing the Long-term Outcome in Primary Spinal Cord Tumors: Review of Literature

Introduction Primary spinal cord tumors are amenable to total surgical excision but the outcomes are still diverse. Objective The purpose of this study is to identify the significant factors which influence the long-term outcome of spinal cord tumors. Materials and Methods A total of 84 consecutive spinal cord tumor patients who underwent microsurgical excision during April 2003 to 2014 were retrospectively studied. Patients operated after April 2014 were not considered. Ten factors were taken and correlated with the outcome. Multiple regression analysis was used. Patients were periodically evaluated at 1, 6 months, 1, 2, and 5 years. In 72 cases, postoperative MRI scans were performed. The follow-up period ranged between 5 to 15 years. Results In our study, we had 64 extramedullary tumors and 21 intramedullary tumors with varied pathology. Axial location of the tumour (20.294/0.000), extent of the resection of the tumour (13.827/0.001), preoperative Nurick grade (11.349/0.023), and location of the tumour in the spine with respect to vertebral segments (8.151/0.017) were significant predictive factors. We had good outcomes in 65 cases and poor outcomes in 19 cases. Conclusion Our results show location of the tumor with respect to axial plane and vertebral segments were the main contributing factors. They influence the extent of excision of the spinal cord tumor. Preoperative neurological status is the fourth factor which independently determines the long-term outcome. It is one of the few papers where patients have been followed-up for a long period ranging from 5 to 15 years.

NCOG-23. PATIENT-REPORTED SYMPTOM BURDEN AND INTERFERENCE: A COMPARISON BETWEEN COVID-19 PANDEMIC YEAR AND NORMATIVE DATA IN PATIENTS WITH CENTRAL NERVOUS SYSTEM (CNS) TUMORS

CNS tumor patients are highly symptomatic causing interference with activity and worse quality of life. Social distancing due to the COVID-19 pandemic increased demands on the patient, caregivers, clinicians, and the health care system. The NCI’s Neuro-Oncology Branch Natural History Study (NHS) systematically collected patient-reported outcomes (PROs) provide insight into how these challenges influenced symptom burden and interference during the COVID year. METHODS: Patient and disease characteristic as well as patient-reported symptoms and interference (MDASI-BT/-SP) and general health status (EQ-5D-3L) from 3/2020-2/2021) were compared to NHS normative sample collected prior to 3/2020. RESULTS: The sample (n = 178) was primarily White (82%), male (55%), median age of 45 (range 18 – 79) and KPS ³ 90 (51%). The majority had high-grade (70%) brain (83%) tumors (BT) with ≥ 1 prior recurrence (60%) and 25% were on active treatment. Clinical visits were primarily conducted via telehealth (64%) and 20% of all patients were diagnosed with progression at the time of assessment. Most commonly reported moderate-severe symptoms among BT patients were fatigue (30%), difficulty remembering (28%), feeling drowsy (22%). Among spinal cord tumor patients, fatigue (39%), pain (35%) and numbness/tingling in arms/legs/trunk (35%) were most frequently reported. These symptoms were reported in similar frequencies by the normative sample. Nearly half of the COVID year sample (48%) reported moderate-severe activity-related interference. Reported problems with mobility (38%), self-care (19%), pain/discomfort (40%), and usual activities (50%) were similar in both groups except for increased mood disturbance (53%) was reported during the COVID year. CONCLUSION: These findings support CNS tumor patients remained highly symptomatic with significant impact on health-related quality of life during the COVID year. Clinicians should develop timely individual care plans to help BT patients navigate their disease course. Evaluation of risk associated with more severe symptoms and functional limitations are ongoing.

Recent advances and new discoveries in the pipeline of the treatment of primary spinal tumors and spinal metastases: A scoping review

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 towards the metastatic population as opposed to the primary tumours 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.

Spinal Cord Diffuse Leptomeningeal Glioneuronal Tumor Presenting without Leptomeningeal Dissemination

<b><i>Background and Importance:</i></b> Diffuse leptomeningeal glioneuronal tumor (DLGNT) represents a provisional entity in the 2016 World Health Organization classification of tumors; it is characterized by a widespread leptomeningeal growth and oligodendroglial-like cytology. To this day, 4 pediatric patients have been reported to present with an isolated spinal cord tumor in the absence of leptomeningeal dissemination. Gross total resection (GTR) was achieved in only 1 patient. We present the clinical and technical nuances of this unique type of tumor, as well as the second reported case of GTR in a patient with DLGNT. <b><i>Clinical Presentation:</i></b> A 4-year-old boy presented to the emergency department after an episode of flaccid paralysis of bilateral lower extremities. MRI showed an intramedullary spinal cord tumor centered at T8. The patient was taken to the operative room, where a laminectomy and tumor resection were performed; cystic and solid tumor components were identified. Pathology report was consistent with DLGNT. After achieving GTR, patient is free of recurrence after a 15-month follow-up. <b><i>Conclusion:</i></b> No standard treatment for DLGNT has been identified. Current literature report surgery and chemotherapy with variable success rates. DLGNT presenting as an isolated intramedullary tumor is an uncommon condition which progression appears to be halted when treated promptly. Identifying solid and cystic components of this tumor is crucial for achieving GTR.

SPASTIC PARAPARESIS IN YOUNG MEN WITH SPINAL MENINGIOMA AND SPINAL ARTERIOVENOUS MALFORMATION: A CASE REPORT

Background: Compared to trauma cases, the publication of non-traumatic events is much less, although the incidence is estimated to be as much as a traumatic event. This case report presents spinal cord tumors together with arteriovenous malformation (AVM) in young men, where there are no previous case reports that the authors can find.Case Report: A 22-year-old male patient with complaints of pain in the back (NPRS 8-9), followed by difficulty moving the lower limb accompanied by a burning sensation, problem in defecation, and urination. We found motor weakness in the lower extremities (1/1), positive pathological reflexes, and sensibility disorders on physical examination.Discussion: According to history taking, physical examination, and MR Imaging, the spastic paraparesis symptom in this patient is likely due to a spinal cord tumor, which is meningioma (according to histopathological examination). Where back pain rarely presents in the patient with spinal AVM. Laminectomy and tumor destruction were the gold standards of therapy, and the symptom was relieved.Conclusion: A complete examination is the main key in determining the appropriate therapy for the patient's disease. In this case, the manifestation of spastic paraparesis was likely to be caused by meningioma rather than spinal AVM. Laminectomy and extirpation of the spinal meningioma were done as the gold standard of therapy, and the symptom was relieved. In contrast, spinal AVM was not performed­ and is still under observation.

Microsurgical resection of a giant cervico-medullary ependymoma: 2D-dimensional video

Background: Ependymoma is a slowly growing benign neoplasm that constitutes 3–9% of all neuroepithelial spinal cord tumors.[3,4] They rarely involve the cervicomedullary junction where they both compress the distal brainstem and upper cervical cord. Due to the critical contiguous structures, gross total resection of these lesions may result in significant morbidity/mortality.[1,2] Utilizing intraoperative neuromonitoring can help limit the risks of removing these lesions. Not when considering the risk/complications of partial versus total resection, the surgeon should keep in mind that they are benign slow growing tumors with relatively good long-term survivals following partial removals. This surgical video shows the surgical strategy and management of a giant cervicomedullary ependymoma performed in a 23-year-old female. Case Description: A 23-year-old female presented with cervical pain and quadriparesis of 1-year’s duration. The MR with/without gadolinium showed a large intradural, intramedullary cervical spinal cord tumor that severely expanded the spinal cord. It contained a significant cystic component, extending from the lower brain stem to the inferior aspect of C7. The lesion was hyperintense on T1 and T2 sequences and demonstrated minimal contrast enhancement. Surgery warranted a posterior cranio-cervical midline approach consisting of a suboccipital craniectomy with laminotomy. The pathological diagnosis was consistent with an ependymoma (WHO I). Fifteen days postoperatively, the patient was discharged with a minimal residual quadriparesis that largely resolved within 6 postoperative months. Three months later, the MRI confirmed complete tumor removal of the lesion. Notably, longer-term follow-up is warranted before complete excision can be confirmed. If there is a recurrence, repeat resection versus stereotactic radiosurgery may be warranted. Conclusion: This video highlights a safe and effective surgical technique for the resection of a giant cervicomedullary ependymoma.

Cervical neuromyelitis optica with thoracic ependymoma

Background: The occurrence of cervical neuromyelitis optica (NMO) in a patient with a thoracic ependymoma is uncommon. Here, we present a patient with a spinal ependymoma who developed the new onset of NMO 2 months later. Case Description: A 66-year-old male presented with right lower limb weakness. The magnetic resonance (MR) revealed an intramedullary spinal cord tumor at the T2-T4 level. It was surgically excised and proved pathologically to be an ependymoma. 2 months later, the patient presented with an acute partial quadriparesis and a high signal intensity cord lesion at the C2-C3 level attributed to seropositive NMO (i.e. additional diagnostic studies confirmed this diagnosis). Conclusion: Patients with intramedullary thoracic ependymomas may also develop NMO resulting in recurrent/ new neurological deficits. Critical studies utilized to diagnose NMO include brain and spine MRs showing unique intramedullary brain/cord lesions, aquaporin-4 positive serology, and classical abnormal visual studies. If the diagnosis of NMO is established, multiple additional medical therapies are warranted.