Spinal Cord Astrocytoma Research Articles

Immunotherapeutic treatments for spinal and peripheral nerve tumors: a primer.

Spinal and peripheral nerve tumors are a heterogeneous group of neoplasms that can be associated with significant morbidity and mortality despite the current standard of care. Immunotherapy is an emerging therapeutic option to improve the prognoses of these tumors. Therefore, the authors sought to present an updated and unifying review on the use of immunotherapy in treating tumors of the spinal cord and peripheral nerves, including a discussion on mechanism of action, drug delivery, current treatment techniques, and preclinical and clinical studies. Current data in the literature regarding immunotherapy were collated and summarized. Targeted tumors included primary and secondary spinal tumors, as well as peripheral nerve tumors. Four primary modalities of immunotherapy (CAR T cell, monoclonal antibody, viral, and cytokine) have been reported to target spine and peripheral nerve tumors. Of the primary spinal tumors, spinal cord astrocytomas had the most preclinical evidence supporting immunotherapy success with CAR T-cell therapy targeting the H3K27M mutation, whereas spinal schwannomas and ependymomas had the most evidence reported for monoclonal antibody therapy preclinically. Of the secondary spinal tumors, primary CNS lymphomas demonstrated some clinical response to immunotherapy, whereas multiple myeloma and bone tumor experiences with immunotherapy were largely limited to concept only. Within peripheral nerve tumors, the use of immunotherapy to treat neurofibromas in the setting of syndromes has been suggested in theory, and possible immunotherapeutic targets have been identified in malignant peripheral nerve tumors. To date, there have been 2 clinical trials involving spine tumors and 2 clinical trials involving peripheral nerve tumors that have reported results, all of which are promising but require validation. Immunotherapy to treat spinal and peripheral nerve tumors has become an emerging area of research and interest. A large amount of preclinical data supporting the translation of this therapy into practice, aimed at ameliorating the poor prognoses of specific tumors, have been reported. Future clinical studies for translation will focus on the optimal therapy type and administration route to best target these tumors, which often preclude total surgical resection given their proximity to the neural and vascular elements of the spine.

Development and Validation of a Personalized Prognostic Prediction Model for Patients With Spinal Cord Astrocytoma.

BackgroundThe study aimed to investigate the prognostic factors of spinal cord astrocytoma (SCA) and establish a nomogram prognostic model for the management of patients with SCA.MethodsPatients diagnosed with SCA between 1975 and 2016 were extracted from the Surveillance, Epidemiology, and End Results (SEER) database and randomly divided into training and testing datasets (7:3). The primary outcomes of this study were overall survival (OS) and cancer-specific survival (CSS). Cox hazard proportional regression model was used to identify the prognostic factors of patients with SCA in the training dataset and feature importance was obtained. Based on the independent prognostic factors, nomograms were established for prognostic prediction. Calibration curves, concordance index (C-index), and time-dependent receiver operating characteristic (ROC) curves were used to evaluate the calibration and discrimination of the nomogram model, while Kaplan-Meier (KM) survival curves and decision curve analyses (DCA) were used to evaluate the clinical utility. Web-based online calculators were further developed to achieve clinical practicability.ResultsA total of 818 patients with SCA were included in this study, with an average age of 30.84 ± 21.97 years and an average follow-up time of 117.57 ± 113.51 months. Cox regression indicated that primary site surgery, age, insurance, histologic type, tumor extension, WHO grade, chemotherapy, and post-operation radiotherapy (PRT) were independent prognostic factors for OS. While primary site surgery, insurance, tumor extension, PRT, histologic type, WHO grade, and chemotherapy were independent prognostic factors for CSS. For OS prediction, the calibration curves in the training and testing dataset illustrated good calibration, with C-indexes of 0.783 and 0.769. The area under the curves (AUCs) of 5-year survival prediction were 0.82 and 0.843, while 10-year survival predictions were 0.849 and 0.881, for training and testing datasets, respectively. Moreover, the DCA demonstrated good clinical net benefit. The prediction performances of nomograms were verified to be superior to that of single indicators, and the prediction performance of nomograms for CSS is also excellent.ConclusionsNomograms for patients with SCA prognosis prediction demonstrated good calibration, discrimination, and clinical utility. This result might benefit clinical decision-making and patient management for SCA. Before further use, more extensive external validation is required for the established web-based online calculators.

Prognostic Factors Independently Associated With Improved Progression-Free Survival After Surgical Resection in Patients With Spinal Cord Astrocytomas: An Institutional Case Series.

Spinal astrocytomas are rare tumors in which the extent of resection and adjuvant therapy remain controversial. A number of new molecular markers are used, but their utility in spinal cord tumors remains unclear. To determine prognostic indicators of progression-free survival (PFS) in patients with spinal astrocytomas. A retrospective chart review identified all patients managed at a single institution for spinal cord astrocytomas between 1999 and 2019. Data collected included baseline demographics, presenting signs and symptoms, tumor size, operative outcomes, and adjuvant treatment. Pathological data including histopathological grade, proliferative index, and molecular profile were collected. Duration of follow-up, presence of tumor progression, and status at last follow-up were documented. Univariate and multivariate analyses were performed to determine predictors of PFS. Seventy-five patients were included in our study with an average age of 42 ± 17 yr. The thoracic spine was the most commonly involved spinal segment (50 patients), and most patients had grade I or grade II tumors (50 patients). On univariate analysis, gross total resection (GTR), lower tumor grade, and low Ki-67 index were associated with lower tumor progression (P-values .01, .04, and .00013, respectively). On multivariate Cox regression analysis, GTR, adjuvant chemotherapy and radiation, and low Ki-67 index were independent predictors of PFS (P-values .009, .011, and .031, respectively). In spinal astrocytomas, GTR, adjuvant therapy, and low Ki-67 are independently associated with improved PFS. These data may help guide management of these tumors and provide important prognosticating information.

Prognostic factors and nomogram prediction of survival probability in primary spinal cord astrocytoma patients.

Knowledge on the management of spinal cord astrocytoma (SCA) remains scarce. Here, the authors constructed and validated a predictive nomogram, often used for individualized prognosis and evaluations, to estimate cancer-specific survival (CSS) and overall survival (OS) for patients with SCA. Epidemiological characteristics were compared between low-grade SCA (LGSCA) and high-grade SCA (HGSCA) patients from the Surveillance, Epidemiology, and End Results (SEER) database. Risk factors for CSS and OS were determined using univariate and multivariate analyses and Kaplan-Meier curves. A nomogram was developed to individually predict the 3-, 5-, and 10-year CSS and OS rates. The clinical usefulness of the nomogram was assessed using calibration plots, the concordance index (C-index), and time-dependent receiver operating characteristic curves. A total of 468 LGSCA and 165 HGSCA patients were eligible for inclusion. LGSCA and HGSCA patients demonstrated differences in age, tumor extension, insurance status, adjuvant treatment, and survival. Multivariate analysis demonstrated that in the LGSCA group, tumor extension, surgery type, and adjuvant therapy were individually associated with CSS. The distance of tumor extension and WHO grade were individually associated with CSS in the HGSCA group. The prognostic variables were further demonstrated using the Kaplan-Meier method, which also suggested that adjuvant treatment provided no advantage to HGSCA patients. A nomogram was constructed, and the C-index for CSS was 0.84 by internal validation (95% CI 0.79-0.90). This research suggests that the distance of tumor extension, type of surgery, and adjuvant therapy are significant risk factors for CSS using multivariate analysis in the LGSCA group. Adjuvant treatment provided no advantages for CSS or OS in patients with HGSCAs. The nomogram may be clinically useful to healthcare providers.

Clinical outcomes and a therapeutic indication of intramedullary spinal cord astrocytoma.

Retrospective cohort study. Although intramedullary astrocytoma is associated with a high mortality rate, the optimal treatment has not reached a consensus. This study aimed at evaluating neurologic function and overall survival rate (OSR) in the treatment of this tumor. The single institution in Japan. This study enrolled 67 subjects who underwent surgical treatment for intramedullary astrocytoma. Demographic, imaging, and surgical information were collected from each participant. Tumors were histologically categorized using the World Health Organization classification, and subjects were divided into low-grade (I and II; n = 40) and high-grade (III and IV; n = 27) groups. Neurologic status was evaluated using the modified McCormick scale (MMS). OSR was assessed using Kaplan-Meier methods. The OSR decreased when the pathological grade increased (p < 0.01). Regarding the therapeutic efficacy for low-grade astrocytomas, subjects who underwent gross total resection (GTR) showed a higher OSR than those who did not (p = 0.02). GTR prevented worsening of MMS score, while non-GTR increased the MMS score (p < 0.01). In the high-grade group, 19 and 10 underwent radiation therapy and chemotherapy, respectively. However, both treatments did not improve OSR. Cordotomy was performed for subjects whose lesional area was at the thoracic level, but the OSR did not significantly increase. The most beneficial therapeutic strategy for low-grade astrocytomas was GTR, whereas that for the high-grade tumors was unclear. Further studies with a larger sample size are warranted to validate the effective treatment for malignant astrocytomas.

Molecular diagnostics helps to identify distinct subgroups of spinal astrocytomas

Primary spinal cord astrocytomas are rare, hence few data exist about the prognostic significance of molecular markers. Here we analyze a panel of molecular alterations in association with the clinical course. Histology and genome sequencing was performed in 26 spinal astrocytomas operated upon between 2000 and 2020. Next-generation DNA/RNA sequencing (NGS) and methylome analysis were performed to determine molecular alterations. Histology and NGS allowed the distinction of 5 tumor subgroups: glioblastoma IDH wildtype (GBM); diffuse midline glioma H3 K27M mutated (DMG-H3); high-grade astrocytoma with piloid features (HAP); diffuse astrocytoma IDH mutated (DA), diffuse leptomeningeal glioneural tumors (DGLN) and pilocytic astrocytoma (PA). Within all tumor entities GBM (median OS: 5.5 months), DMG-H3 (median OS: 13 months) and HAP (median OS: 8 months) showed a fatal prognosis. DMG-H3 tend to emerge in adolescence whereas GBM and HAP develop in the elderly. HAP are characterized by CDKN2A/B deletion and ATRX mutation. 50% of PA tumors carried a mutation in the PIK3CA gene which is seemingly associated with better outcome (median OS: PIK3CA mutated 107.5 vs 45.5 months in wildtype PA). This exploratory molecular profiling of spinal cord astrocytomas allows to identify distinct subgroups by combining molecular markers and histomorphology. DMG-H3 tend to develop in adolescence with a similar dismal prognosis like GBM and HAP in the elderly. We here describe spinal HAP with a distinct molecular profile for the first time.

Concomitant KIAA1549-BRAF fusion and IDH mutation in Pediatric spinal cord astrocytoma: a case report and literature review

Primary tumors of the spinal cord are rare, accounting for 3-6% of tumors in the central nervous system, particularly in children. KIAA1549-BRAF fusion is more common in pilocytic astrocytoma (PA) and IDH1 R132H mutation is rare in infratentorial tumors. Here, we report a 10-year-old male patient who presented with weakness in lower limbs that progressed to difficulty walking. Magnetic resonance imaging (MRI) revealed an intramedullary solid-cystic lesion from the medulla oblongata to the thoracic spin 4 level, with the expansion of the spinal cord. The lesion exhibited patchy enhancement at C4-T1, indicating a tentative diagnosis of astrocytoma. The patient underwent resection of the lesion in the spinal canal from the cervical 6 level to the thoracic 2 level. Histopathology confirmed diagnosis of astrocytoma, WHO grade 2. Genetic analysis showed both IDH1 R132H mutation and KIAA1549-BRAF fusion. Therefore, our integrated diagnosis was astrocytoma, IDH mutation, WHO grade 2. Its molecular analyses include IDH1 R132H mutation and KIAA1549-BRAF fusion. After the operation, the patient did not receive chemo- or radiotherapy, and underwent an aggressive rehabilitation regiment. Follow up 10months later, symptoms improved. To our best knowledge, this is the first case of concomitant IDH mutation and BRAF fusion in pediatric spinal cord astrocytoma.

Immunotherapy: A Potential Approach for High-Grade Spinal Cord Astrocytomas.

Spinal cord astrocytomas (SCAs) account for 6–8% of all primary spinal cord tumors. For high-grade SCAs, the prognosis is often poor with conventional therapy, thus the urgent need for novel treatments to improve patient survival. Immunotherapy is a promising therapeutic strategy and has been used to treat cancer in recent years. Several clinical trials have evaluated immunotherapy for intracranial gliomas, providing evidence for immunotherapy-mediated ability to inhibit tumor growth. Given the unique microenvironment and molecular biology of the spinal cord, this review will offer new perspectives on moving toward the application of successful immunotherapy for SCAs based on the latest studies and literature. Furthermore, we will discuss the challenges associated with immunotherapy in SCAs, propose prospects for future research, and provide a periodic summary of the current state of immunotherapy for SCAs immunotherapy.

Bevasizumab’a İyi Cevap Veren, Radyoterapi ve Temozolomid Tedavisine Yanıtsız Bir Erişkin Spinal Kord Astrositomu; Olgu Sunumu ve Literatür Derlemesi

The incidence of spinal cord astrocytomas (SCAs) is very low and therefore, management of these tumors are challenging due to this paucity. In this report, a patient with a recurrent SCA was successfully treated with bevacizumab after failing to previous therapies including radiotherapy (RT) and temozolomide (TMZ). Bevacizumab is an angiogenesis inhibitor that is widely used in high grade gliomas and shows promise for SCAs spinal cord astrocytomas but there is limited data about the usage of bevacizumab in SCAs. We herein add an important contribution to the literature by reporting a nearly total response to bevacizumab in a patient with low grade SCA.

A comprehensive model including preoperative peripheral blood inflammatory markers for prediction of the prognosis of diffuse spinal cord astrocytoma following surgery

PurposeDue to the rarity of diffuse spinal cord astrocytoma, an effective model is still lacking to stratify their prognosis. Here, we aimed to establish a prognostic model through comprehensively evaluating clinicopathological features and preoperative peripheral blood inflammatory markers in 89 cases.MethodsWe performed univariate and multivariate Cox regression to identify prognosis factors. The Kaplan–Meier curves and ROC curves were employed to compare the prognostic value of selected factors.ResultsIn addition to clinicopathological factors, we revealed the preoperative peripheral blood leukocyte count, neutrophils-to-lymphocytes ratio (NLR), and platelet-to-lymphocyte ratio (PLR) were also significantly correlated with overall survival of spinal cord astrocytoma in univariate Cox regression, and NLR was still significant in multivariate Cox analysis. Further, we demonstrated that NLR ≤ 3.65 and preoperative McCormick score (MMS) ≤ 3 were independently correlated with better survival of WHO grade IV tumors. Meanwhile, Ki-67 < 10% and resection extent ≥ 90% were independent prognostic factors in WHO grade II/III tumors. Finally, we developed a prognostic model that had better predictive efficiencies than WHO grade and histological grade for 1-year (AUC = 76.6), 2- year (AUC = 80.9), and 3-year (AUC = 80.3) survival. This model could classify tumors into 4 classifications with increasingly poor prognosis: 1, WHO grade II/III, with Ki-67 < 10% and resection extent ≥ 90%; 2, WHO grade II/III, Ki-67 ≥ 10% or resection < 90%; 3, WHO grade IV, NLR ≤ 3.65 and MMS ≤ 3; 4, WHO grade IV, with NRL > 3.65 or MMS = 4.ConclusionWe successfully constructed a comprehensive prognostic model including preoperative peripheral blood inflammatory markers, which can stratify diffuse spinal cord astrocytoma into 4 subgroups.

Thoraco-lumbar high grade spinal cord astrocytoma: first documented case in a tertiary medical center and review of literature

&lt;p class="abstract"&gt;This presents the first documented case of high grade spinal cord astrocytoma in a pediatric patient treated at a tertiary medical center and gives a comprehensive review of literature on the most current modes of diagnosis, treatment, and prognosis. We report the case of a 7-year-old male with complaints of inability to ambulate and associated low back pain, which rapidly deteriorated to complete paraplegia. Magnetic resonance imaging (MRI) of his spine revealed an intradural intramedullary spinal cord lesion extending from T11 to L5 vertebra, with considerations of spinal astrocytoma and ependymoma. The patient underwent en bloc laminectomy on T9-L3, partial resection of the intramedullary cord tumor, and subsequent laminoplasty with mini plates and screws. Histopathologic studies showed spinal cord astrocytoma, World Health Organization (WHO) grade 3 (anaplastic type). The patient’s neurologic status did not improve after the surgery and was advised to undergo chemo-radiation therapy but was lost to follow up. Ten months post-operation, patient sought consult due to severe back pain, generalized body weakness, and difficulty of breathing and was then admitted. Patient’s condition worsened that eventually resulted to his demise.&lt;/p&gt;&lt;p class="abstract"&gt; &lt;/p&gt;

Rare Cases of IDH1 Mutations in Spinal Cord Astrocytomas

A low occurrence rate of spinal cord gliomas (4.3% of primary and glial CNS tumors) and the associated difficulties in building statistically significant cohorts of patients considerably slow down the development of effective approaches to the treatment of spinal cord tumors compared to brain tumors. Despite our extensiveknowledge regardingIDHmutations in intracranial tumors, mutations of this gene in spinal cord astrocytomas remain poorly understood. In thisstudy, we report on five cases of identified mutations in theIDH1gene in spinal cord astrocytoma cells, two of which are unique, as they have never been previously described in CNS gliomas.

A refractory case of CDK4-amplified spinal astrocytoma achieving complete response upon treatment with a Palbociclib-based regimen:a case report

BackgroundSpinal cord astrocytoma is a rare neoplasm, and patients usually recur within months after surgery. There is currently a lack of consensus regarding post-operative treatment. Clinical data on the activity of systemic treatment like chemoradiotherapy and anti-angiogenic agents also remained scant. Next-generation sequencing (NGS) -based genomic profiling thus may help identify potential treatment options for a subset of patients that harbor actionable genetic alterations.Case presentationWe reported for the first time a refractory case of grade III spinal cord astrocytoma that underwent two surgeries but eventually progressed following post-operative chemoradiotherapy plus bevacizumab. Hybridization capture-based NGS using a 381-gene panel disclosed cyclin dependent kinase 4 (CDK4) amplification and after receiving a triplet regimen containg palbociclib for 15 months, the patient achieved complete response.ConclusionsThis case demonstrated the importance of genetic profiling and the benefit of a multi-modality treatment strategy in cancer management.

Exploring the Feasibility and Effects of a Ketogenic Diet in Patients With CNS Malignancies: A Retrospective Case Series.

Background: Recently, the ketogenic diet has been proposed as an adjunct treatment for a range of medical conditions including weight loss, diabetes, cancer, and neurodegenerative diseases. Because malignant CNS tumors are highly dependent on glucose, the use of a ketogenic diet as an adjunct therapy is currently being explored. This case series summarizes our experience implementing a ketogenic diet for patients with CNS malignancies.Methods: Patients diagnosed with CNS malignancies following a ketogenic diet were identified between 2015 and 2017. Malignancies included confirmed diagnoses of glioblastoma (GBM), astrocytoma, or oligodendroglioma. With guidance from a registered dietitian, ketone levels, glucose levels, and weight were regularly collected for several patients along with patient-reported symptoms and adverse effects. Interested patients were asked to follow a 3:1 ketogenic diet for 120 days. The ketogenic diet is a high-fat, moderate protein, and very low carbohydrate diet, where patients limited carbohydrate intake to ≤20 g per day. Brain imaging was reviewed. A series of descriptive analyses were conducted.Results: The ketogenic diet was initiated in 12 patients of which 8 patients contributed data on their blood glucose and ketone levels. The majority of patients were male (n = 10) with a median age of 45 (range 32–62). Diagnoses included GBM (n = 6), grade 2/3 astrocytomas (n = 5) and one patient with a grade 2 spinal cord astrocytoma. Ten of the 12 patients were receiving concurrent treatment; two received supportive care only. The majority of patients with evaluable data (n = 8) maintained ketone levels above 0.5 mM for the duration of 120-day period. Ketone levels generally increased from baseline while glucose levels and BMI decreased. Overall, patients reported improved symptoms over the course of the diet. Imaging also suggested improved disease control and reduction in vasogenic edema.Conclusion: Taking advantage of a tumor’s metabolic inflexibility can have a positive impact on patients, particularly those with CNS malignancies. More structured and statistically planned clinical trials are needed to determine the margin of impact of a ketogenic diet.

The molecular characteristics of spinal cord gliomas with or without H3 K27M mutation

Due to the rare incidence of spinal cord astrocytomas, their molecular features remain unclear. Here, we characterized the landscapes of mutations in H3 K27M, isocitrate dehydrogenase 1 (IDH1) R132H, BRAF V600E, and the TERT promoter in 83 diffuse spinal cord astrocytic tumors. Among these samples, thirty-five patients had the H3 K27M mutation; this mutant could be observed in histological grade II (40%), III (40%), and IV (20%) astrocytomas. IDH1 mutations were absent in 58 of 58 cases tested. The BRAF V600E mutation (7/57) was only observed in H3-wildtype astrocytomas, and was associated with a better prognosis in all histological grade II/III astrocytomas. TERT promoter mutations were observed in both H3 K27M-mutant (4/25) and -wildtype (9/33) astrocytomas, and were associated with a poor prognosis in H3-wildtype histological grade II/III astrocytomas. In the 2016 WHO classification of CNS tumors, H3 K27M-mutant diffuse midline gliomas, including spinal cord astrocytomas, are categorized as WHO grade IV. Here, we noticed that the median overall survival of histological grade II/III H3 K27M-mutant cases (n = 28) was significantly longer than that of either the total histological grade IV cases (n = 12) or the H3 K27M-mutant histological grade IV cases (n = 7). We also directly compared H3 K27M-mutant astrocytomas to H3-wildtype astrocytomas of the same histological grade. In histological grade II astrocytomas, compared to H3-wildtype cases (n = 37), H3 K27M-mutant patients (n = 14) had showed a significantly higher Ki-67-positive rate and poorer survival rate. However, no significant differences in these parameters were observed in histological grade III and IV astrocytoma patients. In conclusion, these findings indicate that spinal cord astrocytomas are considerably different from hemispheric and brainstem astrocytomas in terms of their molecular profiles, and that the histological grade cannot be ignored when assessing the prognosis of H3 K27M-mutant spinal cord astrocytomas.

Application of intraoperative ultrasound in microsurgical treatment of intramedullary ependymoma and astrocytoma of the spinal cord

Objective To evaluate the role of intraoperative ultrasound (IoUS) in the microsurgical treatment of intramedullary ependymoma and astrocytoma of the spinal cord. Methods A retrospective review was performed on 78 cases of intramedullary ependymoma and 34 cases of intramedullary astrocytoma which were treated microsurgically between January 2010 and May 2018 at Department of Neurosurgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology. All cases of ependymoma or astrocytoma were divided into IoUS group(44 cases of ependymomas, 18 cases of astrocytomas) and control group(34 cases of ependymomas, 16 cases of astrocytomas) according to whether IoUS was employed during operation. All patients were followed up in the clinic or by telephone to assess tumor recurrence and spinal function recovery by radiographic reexamination and Modified McCormick Scale (MMS), which were then compared between IoUS and control group in ependymomas and astrocytomas, respectively. The accuracy of intraoperative ultrasound in evaluating the gross total resection (GTR) rate was also evaluated. Results No significant differences were observed in age, sex, presenting symptoms, MMS or spinal cord segment of tumor location between IoUS and control groups in ependymoma or astrocytoma (all P>0.05). Compared with control group in ependymoma, the IoUS group had slightly higher GTR rate [97.7% (43/44) vs. 91.2% (31/34)], slightly higher rate of good outcomes in terms of spinal function [36.4% (16/44) vs. 32.4% (11/34)] and slightly lower incidence of complications [8.8% (3/44) vs. 11.8% (4/34)], which, however, were not significantly different (all P>0.05). For astrocytoma, the IoUS group had significantly higher GTR rate [16/18 vs. 9/16, P 0.05] and slightly lower incidence of complications [0/18 vs. 2/16, P>0.05] compared with control group. However, those differences were not significant (P>0.05). Postoperative enhanced MRI was used as the gold standard for verification of GTR. The accuracy rate of IoUS for prediction of GTR was 97.7% (43/44) in ependymoma and 14/16 in astrocytoma. Conclusion The intraoperative ultrasound technique could facilitate the real-time and accurate judgement of tumor resection extent, which might have greater application value in the astrocytoma. Key words: Ependymoma; Astrocytoma; Microsurgery; Intraoperative ultrasound; Intrame-dullary tumor

MICROSURGICAL MANAGEMENT OF LOW-GRADE SPINAL CORD ASTROCYTOMA IN ADULTS: A PERSONAL CASE SERIES REPORT AND BRIEF LITERATURE REVIEW.

SUMMARYAstrocytoma is the second most common intramedullary tumor of predominantly low-grade malignancy in adult patients. Adult astrocytomas have better-quality prognosis compared with astrocytomas in children. Although a standardized surgical management protocol for spinal cord glioma is currently unavailable, surgery of low-grade astrocytoma should be aimed at gross total resection to preserve neurological function and to improve the outcome. Herein, we present a personal case series of four consecutive adult spinal cord astrocytoma patients who were operated on during the last few years. Tumor resection was performed in all patients utilizing microsurgical technique and intraoperative neurophysiologic monitoring. We also provide a literature review of the treatment of intramedullary astrocytoma in adults and discuss contemporary surgical management and prognosis.

Rare Cases of IDH1 Mutations in Spinal Cord Astrocytomas.

A low occurrence rate of spinal cord gliomas (4.3% of primary and glial CNS tumors) and the associated difficulties in building statistically significant cohorts of patients considerably slow down the development of effective approaches to the treatment of spinal cord tumors compared to brain tumors. Despite our extensive knowledge regarding IDH mutations in intracranial tumors, mutations of this gene in spinal cord astrocytomas remain poorly understood. In this study, we report on five cases of identified mutations in the IDH1 gene in spinal cord astrocytoma cells, two of which are unique, as they have never been previously described in CNS gliomas.

Intracranial Metastases of Intramedullary Spinal Cord Low-Grade Astrocytoma

AbstractThis paper reports a case of intramedullary spinal cord low-grade (LG) astrocytoma that developed brain metastases after 21 months. A 6-year-old child presented with lower spine pain and falls during daily activity. A spinal cord mass was detected using spinal magnetic resonance imaging (MRI), and brain MRI was normal. The spinal lesion was partially resected, and pathological findings revealed LG astrocytoma (WHO Grade II). The patient underwent thoracolumbar radiotherapy. He returned 21 months following initial admission with symptoms of nausea, vomiting, headaches, and seizure. Brain MRI revealed multiple intracranial masses at the posterior fossa, left lateral ventricle, cerebellopontine angles, and left Meckel cave. A recurrent lesion was detected in the thoracic and lumbar regions of the spinal cord and in the cauda equina. The patient underwent chemotherapy. This rare case warns practitioners to monitor closely the cases of spinal cord astrocytoma that are diagnosed as LG tumors based on histology.

Microsurgical Resection of Low-Grade Spinal Cord Astrocytoma: 2-Dimensional Operative Video.

This video demonstrates microsurgical resection of low-grade (Grade I) thoracic spinal cord astrocytoma. Astrocytomas are the most common pediatric intramedullary tumors and occur secondary to ependymomas in adults.1,2 The treatment goal should be radical gross total resection while preserving neurological function with intraoperative neuromonitoring.2-4 To our knowledge, this is the first video report on microsurgical resection of low-grade spinal cord astrocytoma. A 58-yr-old female presented with gait disturbances has been featured in this video. A spinal MRI revealed T1/T2 hyperintense intramedullary tumor at T4, suggesting spinal cord astrocytoma versus ependymoma. Dura was opened in the midline and tacked to the surrounding muscle tissue with tacking sutures. The arachnoid was opened separately with microscissors, keeping it intact for closure at the end of surgery. Liga clips were applied to hold the arachnoid to the dura. Myelotomy was done in the midline,5 opening the posterior median sulcus over the length of the tumor and extending proximally and distally a few millimeters beyond the tumor.3,5 The tumor reduction was achieved by coagulation or debulking. Once sufficient debulking was achieved, dissection of the tumor margins toward surrounding cord was performed. Preoperative recording of motor-evoked potentials (MEPs) compared with continuous intraoperative MEPs were essential for preserving the neurological function and quality of life. The intraoperative finding of a clear tumor plane of resection carries positive prognostic significance and reduces the risk of complications.2,3,6 A pathohistological diagnosis showed a low-grade (Grade I) spinal cord astrocytoma (pilocytic). Follow-up MRI showed no sign of tumor or recurrence. The patient recovered fully and was neurologically intact.Written consent was obtained directly from the patient.