Pediatric Intramedullary Spinal Cord Tumors Research Articles

78. An NCDB analysis of pediatric intramedullary spinal cord tumors

78. An NCDB analysis of pediatric intramedullary spinal cord tumors

Pediatric intramedullary spinal cord tumors: A national cancer database analysis of demographics, patterns of care, and survival

ObjectiveQuery the National Cancer Database (NCDB) to delineate epidemiologic frequency, care patterns, and survival outcomes of pediatric intramedullary spinal cord tumors (IMSCTs). MethodsIMSCTs included ependymoma, astrocytoma, and hemangioblastoma. We examined data from the NCDB spanning 2004–2018, focusing on IMSCT in children aged 0–21 years. Our analysis included logistic and Poisson regression, Kaplan-Meier survival estimates, and Cox proportional hazards models. ResultsThis study included 1066 patients aged 0–21 years. 59.4 % of patients were male, while 83.1 % were white. The most common tumor histology was ependymoma (57.5 %), followed by astrocytoma (36.1 %) and hemangioblastoma (6.4 %). 24.9 % of patients received radiotherapy, with radiotherapy utilization being highest among patients aged 6–10 years. Chemotherapy utilization was highest in patients aged 0–5 years. 87.2 % of patients underwent surgical resection, with higher rates in patients aged 16–21 years. Overall survival did not differ significantly between resected and non-resected patients (p = 0.315). Patients in rural areas had worse OS than those in metro areas (HR = 4.42, p = 0.048). Patients with astrocytoma had worse OS compared to other histologies (HR = 2.21, p = 0.003). Astrocytoma patients were over twice as likely to have prolonged LOS compared to ependymoma patients (OR = 2.204, p < 0.001). ConclusionsIn summary, our analysis utilizing the NCDB database provides a comprehensive overview of demographics, care patterns, and outcomes for the largest cohort of pediatric IMSCTs to date. These insights underscore the complexity of managing IMSCTs and emphasize the need for tailored approaches to improve patient outcomes.

Epidemiology and Comparative Analysis of Outcomes of Intramedullary Spinal Cord Tumor Between Pediatric and Adult Patients.

Clinical retrospective study. We sought to evaluate the characteristics of Pediatric intramedullary spinal cord tumors (PISCTs) and to identify differences between pediatric and adult intramedullary spinal cord tumors. Pediatric intramedullary spinal cord tumors (PISCTs) represent a rare clinical entity with limited evidence-base in the literature. This study is a subanalysis of the retrospective multicenter observational study authorized by the Neurospinal Society of Japan, including consecutive patients with spinal intramedullary tumor treated surgically at 58 institutions between 2009 and 2020. Data on 1080 intramedullary spinal cord tumors were obtained, consisting of 91 pediatric and 939 adult patients. Survival was compared using Cox hazard regression while clinical differences were evaluated using multivariable logistic regression that controlled for confounders. Pediatric patients had a shorter overall, and progression-free, survival than adults. Pediatric patients with ISCTs were likely to have scoliosis (odds ratio [OR] = 6.49, 95% confidence interval [CI]: 2.26-18.7), short preoperative symptom duration (OR = 0.99, 95% CI: 0.98-0.99), lower incidence of paresthesia (OR = 0.41, 95% CI: 0.22-0.77), higher incidence of paresis (OR = 2.10, 95% CI: 1.01-4.35), histopathology of astrocytoma (OR = 2.97, 95% CI: 1.19-7.43), and postoperative functional deterioration upon discharge (OR = 2.83, 95% CI: 1.43-5.58). Age was not a statistically significant prognostic factor of overall survival among the pediatric cohort. We found that the clinical characteristics of PISCTs differed between pediatric and adult patients. In terms of histopathological types, astrocytoma was most common in pediatric patients. ISCT occurring at an early age may not be an indicator for poor prognosis.

Role of resection and adjuvant therapy in long-term disease outcomes for low-grade pediatric intramedullary spinal cord tumors.

OBJECTIVE Surgical excision is the mainstay treatment for resectable low-grade intramedullary spinal cord tumors (IMSCTs) in the pediatric age group. Chemotherapy and radiation treatments are generally reserved for progressive or recurrent disease. Given the indolent nature of low-grade tumors and the potential side effects of these approaches, their long-term treatment benefits are unclear. The aim of the study was to determine long-term disease outcomes and the therapeutic roles of surgery and adjuvant therapies in pediatric patients with low-grade IMSCTs over an extended follow-up period. METHODS Case records for all pediatric patients (< 21 years of age) with a histopathological diagnosis of low-grade IMSCT were selected over a period from January 1975 to January 2010. Outcome variables including McCormick functional grade, overall survival (OS), and progression-free survival (PFS) were analyzed with respect to demographic and treatment variables. RESULTS Case records of 37 patients with low-grade IMSCTs were identified, with a mean follow-up duration of 12.3 ± 1.4 years (range 0.5-37.2 years). Low-grade astrocytomas were the most prevalent histological subtype (n = 22, 59%). Gross-total resection (GTR) was achieved in 38% of patients (n = 14). Fusion surgery was required in 62% of patients with pre- or postoperative deformity (10 of 16). On presentation, functional improvement was observed in 87% and 46% of patients in McCormick Grades I and II, respectively, and in 100%, 100%, and 75% in Grades III, IV, and V, respectively. Kaplan-Meier PFS rates were 63% at 5 years, 57% at 10 years, and 44% at 20 years. OS rates were 92% at 5 years, 80% at 10 years, and 65% at 20 years. On multivariate analysis, shunt placement (hazard ratio [HR] 0.33, p = 0.01) correlated with disease progression. There was a trend toward improved 5-year PFS in patients who received adjuvant chemotherapy and radiation therapy (RT; 55%) compared with those who did not (36%). Patients who underwent subtotal resection (STR) were most likely to undergo adjuvant therapy (HR 7.86, p = 0.02). CONCLUSIONS This extended follow-up duration in patients with low-grade IMSCTs beyond the first decade indicates favorable long-term OS up to 65% at 20 years. GTR improved PFS and was well tolerated with sustained functional improvement in the majority of patients. Adjuvant chemotherapy and RT improved PFS in patients who underwent STR. These results emphasize the role of resection as the primary treatment approach, with adjuvant therapy reserved for patients at risk for disease progression and those with residual tumor burden.

Pediatric intramedullary spinal cord tumors: A single center experience

AimTo evaluate clinical and radiological findings, pathological features and treatment modalities in pediatric patients with intramedullary spinal cord tumors. Patients and methodsThe medical records of 36 patients with intramedullary spinal tumors were reviewed for clinical, radiological and histopathological data, chemotherapy, radiotherapy, surgical resection, treatment responses, events, and final outcome. Survival analyses were performed. ResultsThe median age was 7.9 years (range: 1–16 years; male/female ratio:1.4). Majority of the tumors were histopathologically diagnosed as astrocytomas (n = 16, 44.4%) and ependymomas (n = 19, 52.8%); whereas one was unclassified glioma. Overall, 94% of the astrocytomas and 84% of the ependymomas were low-grade, only three tumors were high-grade. In one patient with ependymoma, histopathological grade was undetermined. The primary tumor was commonly located in thoracic (47%) and cervical segments (28%). All patients had undergone surgery (gross-total resection, 33%; subtotal resection, 45%; biopsy, 22%). Radiotherapy was administered to 26 patients (72%) and chemotherapy to 15 patients (42%). The 3-, 5- and 10-year overall survival rates were 72%, 63% and 56%, respectively; and event-free survival rates were 43%, 40% and 40%. Survival did not significantly differ with gender, age groups, lag-time, neurologic status, histopathological tumor type, tumor location, extent of resection, treatment, or treatment responses in univariate survival analyses. Survival rates were significantly higher in patients with low-grade tumors and in ependymoma patients with resected tumors. ConclusionsPatients with low-grade tumors and those who underwent gross-total tumor resection had better prognosis. Surgery remains the main treatment in intramedullary spinal tumors. The role of radiotherapy and chemotherapy is limited and even controversial in low-grade tumors.

Long-term disease and neurological outcomes in patients with pediatric intramedullary spinal cord tumors.

Radical resection is recommended as the first-line treatment for pediatric intramedullary spinal cord tumors (IMSCTs), but it is associated with morbidity, including risk of neurological decline and development of postoperative spinal deformity. The authors report long-term data on clinical and treatment determinants affecting disease survival and neurological outcomes. Case records for pediatric patients (< 21 years of age at presentation) who underwent surgery for IMSCTs at the authors' institution between January 1975 and January 2010 were analyzed. The patients' demographic and clinical characteristics (including baseline neurological condition), the treatment they received, and their disease course were reviewed. Long-term disease survival and functional outcome measures were analyzed. A total of 55 patients (30 male and 25 female) were identified. The mean duration of follow-up (± SEM) was 11.4 ± 1.3 years (median 9.3 years, range 0.2-37.2 years). Astrocytomas were the most common tumor subtype (29 tumors [53%]). Gross-total resection (GTR) was achieved in 21 (38%) of the 55 patients. At the most recent follow-up, 30 patients (55%) showed neurological improvement, 17 (31%) showed neurological decline, and 8 (15%) remained neurologically stable. Patients presenting with McCormick Grade I were more likely to show functional improvement by final follow-up (p = 0.01) than patients who presented with Grades II-V. Kaplan-Meier actuarial tumor progression-free survival rates at 5, 10, and 20 years were 61%, 54%, and 44%, respectively; the overall survival rates were 85% at 5 years, 74% at 10 years, and 64% at 20 years. On multivariate analysis, GTR (p = 0.04) and tumor histological grade (p = 0.02) were predictive of long-term survival; GTR was also associated with improved 5-year progression-free survival (p = 0.01). The prognosis for pediatric IMSCTs is favorable with sustained functional improvement expected in a significant proportion of patients on long-term follow-up. Long-term survival at 10 years (75%) and 20 years (64%) is associated with aggressive resection. Gross-total resection was also associated with improved 5-year progression-free survival (86%). Hence, the treatment benefits of GTR are sustained on extended follow-up.

Long-term incidence and risk factors for development of spinal deformity following resection of pediatric intramedullary spinal cord tumors.

Spinal deformity in pediatric patients with intramedullary spinal cord tumors (IMSCTs) may be either due to neurogenic disability or due to secondary effects of spinal decompression. It is associated with functional decline and impairment in health-related quality-of-life measures. The authors sought to identify the long-term incidence of spinal deformity in individuals who had undergone surgery for IMSCTs as pediatric patients and the risk factors and overall outcomes in this population. Treatment records for pediatric patients (age < 21 years) who underwent surgical treatment for histology-proven primary IMSCTs between 1975 and 2010 were reviewed. All patients were evaluated in consultation with the pediatric orthopedics service. Clinical records were reviewed for baseline and follow-up imaging studies, surgical fusion treatment, and long-term skeletal and disease outcomes. The authors identified 55 patients (30 males and 25 females) who were treated for pediatric IMSCTs between January 1975 and January 2010. The mean duration of follow-up (± SEM) was 11.4 ± 1.3 years (median 9.3 years, range 0.2-37.2 years). Preoperative skeletal deformity was diagnosed in 11 (20%) of the 55 patients, and new-onset postoperative deformity was noted in 9 (16%). Conservative management with observation or external bracing was sufficient in 8 (40%) of these 20 cases. Surgical fusion was necessary in 11 (55%). Posterior surgical fusion was sufficient in 6 (55%) of these 11 cases, while combined anterior and posterior fusion was undertaken in 5 (45%). Univariate and multivariate analysis of clinical and surgical treatment variables indicated that preoperative kyphoscoliosis (p = 0.0032) and laminectomy/laminoplasty at more than 4 levels (p = 0.05) were independently associated with development of spinal deformity that necessitated surgical fusion. Functional scores and 10-year disease survival outcomes were similar between the 2 groups. Long-term follow-up is essential to monitor for delayed development of spinal deformity, and regular surveillance imaging is recommended for patients with underlying deformity. The authors' extended follow-up highlights the risk factors associated with development of spinal deformity in patients treated for pediatric IMSCTs. Surgical fusion allows patients who develop progressive deformity to achieve long-term functional and survival outcomes comparable to those of patients who do not develop progressive deformity.

Management of Pediatric Spinal Cord Astrocytomas: Outcomes With Adjuvant Radiation

Pediatric intramedullary spinal cord tumors are exceedingly rare; in the United States, 100 to 200 cases are recognized annually, of these, most are astrocytomas. The purpose of this study is to report the outcomes in pediatric patients with spinal cord astrocytomas treated at a tertiary care center. An institutional review board-approved retrospective single-institution study was performed for pediatric patients with spinal cord astrocytomas treated at our hospital from 1990 to 2010. The patients were evaluated on the extent of resection, progression-free survival (PFS), and development of radiation-related toxicities. Kaplan-Meier curves and multivariate regression model methods were used for analysis. Twenty-nine patients were included in the study, 24 with grade 1 or 2 (low-grade) tumors and 5 with grade 3 or 4 (high-grade) tumors. The median follow-up time was 55 months (range, 1-215 months) for patients with low-grade tumors and 17 months (range, 10-52 months) for those with high-grade tumors. Thirteen patients in the cohort received chemotherapy. All patients underwent at least 1 surgical resection. Twelve patients received radiation therapy to a median radiation dose of 47.5 Gy (range, 28.6-54.0 Gy). Fifteen patients with low-grade tumors and 1 patient with a high-grade tumor exhibited stable disease at the last follow-up visit. Acute toxicities of radiation therapy were low grade, whereas long-term sequelae were infrequent and manageable when they arose. All patients with low-grade tumors were alive at the last follow-up visit, compared with 1 patient with a high-grade tumor. Primary pediatric spinal cord astrocytomas vary widely in presentation and clinical course. Histopathologic grade remains a major prognostic factor. Patients with low-grade tumors tend to have excellent disease control and long-term survival compared to those with high-grade tumors. This experience suggests that radiation therapy may enhance tumor control with an acceptably low risk of long-term sequelae in this sensitive patient population.

A retrospective analysis of the patterns of failure in pediatric myxopapillary ependymoma

Objective Myxopapillary ependymoma (MPE) is a rare tumor, accounting for 10–20 % of pediatric intramedullary spinal cord tumors. Our objective was to study the patterns of dissemination and failure in pediatric MPE.

Malignant transformation in pediatric spinal intramedullary tumors: case-based update

In children, intramedullary spinal cord neoplasms are rare. These are typically low-grade neuroepithelial tumors, most commonly astrocytomas, ependymomas, and gangliogliomas. Malignant transformation, while common in recurrent adult low-grade gliomas, is an unusual event in pediatric low-grade neoplasms, specifically in intramedullary spinal cord tumors. We report two cases of malignant transformation in low-grade neuroepithelial tumors of the pediatric intramedullary spinal cord. Two children with intramedullary tumors, one with a WHO grade I ganglioglioma and one with a low-grade astrocytoma, were treated surgically, diagnosed histologically, and followed through the course of their disease. Both patients' tumors transformed to higher grades without prior irradiation or chemotherapy, and without a genetic predisposition to tumorigenesis. Malignant transformation can occur in low-grade intramedullary neoplasms in children. This is a novel documented event for pediatric intramedullary spinal cord tumors and a rare event for all pediatric low-grade neuroepithelial tumors without induction by irradiation. A survey of the relevant literature reveals an underwhelming number of studies focusing on malignant transformation in children's CNS tumors relative to adults. Further investigation into molecular mechanisms of pediatric low-grade neoplasms may reveal more aggressive tumor sub-variants predisposed to malignant degeneration.

The Role of Concurrent Fusion to Prevent Spinal Deformity After Intramedullary Spinal Cord Tumor Excision in Children

Spinal deformity is a common development after laminectomy and resection of pediatric intramedullary spinal cord tumors. Our objective is to compare the occurrence of postlaminectomy spinal deformity in children with intramedullary spinal cord tumors that underwent decompression with fusion at the time of surgery to those that did not undergo fusion. A retrospective chart review of 255 children with spinal cord tumors treated at 2 tertiary pediatric cancer centers between was performed. Of these, 52 patients with a biopsy-proven intramedullary spinal cord tumor had complete clinical records and radiographic data. Preoperative spinal alignment, surgical treatment, postoperative deformity, and risk factors for deformity were evaluated. All patients had at least 2-year follow-up. There were 18 females and 34 males with an average age of 8.1 ± 4.1 years. The average time to latest follow-up was 7.6 ± 5.3 years. Moderate or severe postresection spinal deformity (scoliosis > 25 degrees and/or sagittal plane abnormality > 20 degrees requiring bracing or surgery) developed in 57% (21/37) of resections without fusion (laminectomy or laminoplasty alone), and in 27% (4/15) of those with fusion (P = 0.05). Among skeletally mature children, 18 of 28 (64%) developed deformity after laminectomies and laminoplasties, compared with 22% (2/9) of the patients in the fusion group (P = 0.03). Removal of >3 lamina (P = 0.04) was associated with development of postoperative deformity. In the surgical treatment of patients with intramedullary spinal cord tumors, those that undergo instrumentation or in situ fusion at the time of spinal cord tumor excision are significantly less likely to develop postresection spinal deformity. 3, Retrospective comparative study.

Correlation of a preoperative grading scale with progressive spinal deformity following surgery for intramedullary spinal cord tumors in children

Postoperative progressive spinal deformity often complicates functional outcome after resection of pediatric intramedullary spinal cord tumors (IMSCTs). The authors propose a preoperative grading scale that correlates with the postoperative development of progressive spinal deformity requiring subsequent fusion. The data obtained in 164 patients who underwent resection of an IMSCT at a single institution were retrospectively collected and analyzed to determine the development of progressive spinal deformity requiring fusion. A grading scale (range of scores I-V) was created based on the presence or absence of 4 preoperative variables: preoperative scoliosis, involvement of the thorocolumbar junction, age<13 years, and number of surgeries for an IMSCT. The grading scale was then retrospectively applied to this series of 164 children to assess the correlation of variables with subsequent spinal deformity. Nine patients presented with Grade I status, 41 patients with Grade II, 58 patients with Grade III, 44 patients with Grade IV, and 12 patients with Grade V. Overall, 44 patients (27%) developed progressive spinal deformity requiring fusion at a mean follow-up of 5 years after surgery. A higher preoperative grade was associated with an increasing need for subsequent fusion for progressive spinal deformity (Grade I [0%], Grade II [5%], Grade III [26%], Grade IV [40%], and Grade V status [75%]). Application of this grading scheme to a series of resected pediatric IMSCTs has demonstrated its correlation with the incidence of postoperative progressive spinal deformity requiring fusion. The application of a standardized grading scheme will assist in the process of surgical decision making and postoperative evaluation.

Resection of intramedullary spinal cord tumors in children: assessment of long-term motor and sensory deficits

With modern surgical advances, radical resection of pediatric intramedullary spinal cord tumors (IMSCTs) can be expected to preserve long-term neurological function. Nevertheless, postoperative neurological decline is not uncommon after surgery, and many patients continue to experience long-term dysesthetic symptoms. Preoperative predictors of postoperative neurological decline and sensory syndromes have not been investigated and may serve as a guide for surgical risk stratification. Neurological function (as determined using the modified McCormick Scale [mMS]) preoperatively, postoperatively, and 3 months after surgery was retrospectively recorded from patient charts in 164 consecutive patients undergoing resection of IMSCTs. A median 4 years (interquartile range [IQR] 1-8 years) after surgery, long-term motor and sensory symptoms were assessed by telephone interviews and corroborated by subsequent medical visits in 120 available patients. This long-term assessment was retrospectively reviewed for the purposes of this study. The authors reviewed this series to assess long-term motor, sensory, and urinary outcomes and to determine independent risk factors of postoperative neurological decline and long-term sensory dysfunction. Patients were 8.6 +/- 5.7 years old and presented with a median mMS of 2 (IQR 2-4). Three months after surgery, 38 patients (23%) continued to experience decreased neurological function (1 mMS point) incurred perioperatively. Increasing age (p = 0.028), unilateral symptoms (p = 0.046), and urinary dysfunction at presentation (p = 0.004) independently predicted persistent 3-month perioperative decline. At long-term follow-up (median 4 years), 39 (33%) exhibited improvements in their mMS scores, 13 (30%) had improvement in their urinary dysfunction, and 27 (30%) had resolution of their dysesthesias. Seventy-eight patients (65%) experienced long-term dysesthetic symptoms. Increasing age (p = 0.024), preoperative symptom duration > 12 months (p = 0.027), and worsened postoperative mMS score at hospital discharge (p = 0.013) independently increased the risk of long-term dysesthesias. In the authors' experience, nearly one third of patients may experience improvement in motor, sensory, and urinary dysfunction years after IMSCT resection, whereas the majority will continue to experience long-term dysesthetic symptoms. Improvement in motor deficits preceded improvement in sensory syndromes, and urinary dysfunction typically resolved much longer after surgery. The risk of persistent perioperative motor decline was increased with older age, unilateral symptoms, preoperative urinary symptoms, and less severe preoperative neurological deficit. The risk of long-term dysesthesias was increased with older age, increased duration of symptoms prior to resection, and greater postoperative neurological deficit.

Risk factors for progressive spinal deformity following resection of intramedullary spinal cord tumors in children: an analysis of 161 consecutive cases

Gross-total resection of pediatric intramedullary spinal cord tumors (IMSCTs) can be achieved in the majority of cases, with preservation of long-term neurological function. However, progressive spinal deformity requiring subsequent fusion occurs in many cases. It remains unknown which subgroups of patients have the greatest risk for progressive spinal deformity. Data for 161 patients undergoing resection of IMSCTs at a single institution were retrospectively collected and analyzed with regard to the development of progressive spinal deformity requiring fusion and patient functional status (based on the modified McCormick Scale [mMS] and Karnofsky Performance Scale [KPS]) by conducting telephone interviews corroborated by medical records. The independent association of all clinical, radiographic, and operative variables to subsequent progressive spinal deformity was assessed using multivariate logistic regression analysis. Patients were a mean of 8.6 +/- 5.7 years old at the time of surgery. The tumor spanned a mean of six +/- three spinal levels. Preoperative scoliotic deformity was present in 56 cases (35%). Seventy-six patients (47%) had undergone a previous biopsy procedure, and 28 (17%) a prior resection. Gross-total resection (> 95%) was achieved in 122 cases (76%). A median of 9 years (range 1-21) after surgery, progressive spinal deformity requiring fusion developed in 43 patients (27%). The median functional scores at the last follow-up were worse in patients who required fusion compared with those who did not (mMS: 3 compared with 2, p = 0.006; KPS: 80 compared with 90, p = 0.04) despite similar mMS scores between the groups at 3 months postoperatively. An age less than 13 years, preoperative scoliotic deformity (Cobb angle > 10 degrees), involvement of the thoracolumbar junction, and tumor-associated syrinx independently increased the odds of a postoperative progressive deformity requiring fusion 4.4-, 3.2-, 2.6-, and 3.4-fold, respectively (p < 0.05). Each subsequent resection increased the odds of a progressive deformity 1.8-fold (p < 0.05). Symptoms lasting less than 1 month before resection decreased the odds of spinal deformity requiring fusion ninefold (p < 0.05). Progressive spinal deformity requiring fusion occurred in 27% of children undergoing resection of an IMSCT and was associated with a decreased functional status. Preoperative scoliotic deformity, an increasing number of resections, an age less than 13 years, tumor-associated syrinx, and surgery spanning the thoracolumbar junction increased the risk for progressive spinal deformity. Patients possessing one or more of these characteristics should be monitored closely for progressive spinal deformity following surgery.

Spinal deformity and pediatric intramedullary spinal cord tumors

Spinal deformity and pediatric intramedullary spinal cord tumors

Neurological outcome after resection of intramedullary spinal cord tumors in children

With modern surgical advances, radical resection of pediatric intramedullary spinal cord tumors (IMSCT) can be achieved with preservation of long-term neurological function. Clinical and radiographic risk factors predictive of postoperative neurological outcome may serve as a guide for surgical risk stratification. We prospectively reviewed the outcomes of 16 consecutive cases of pediatric IMSCT resection at a single institution. Clinical, radiographic, and operative variables were analyzed as predictors of postoperative neurological function defined by the modified McCormick score (MMS). Sixteen children 10 +/- 5 years old presented with median (interquartile range) MMS score of 2 (1-2) with IMSCTs (eight cervical, eight thoracic) involving 4 +/- 2 levels. Pathology revealed astrocytoma in 12 cases (three pilocytic, four grade II, three grade III, two GBM), gangliogliomas in two, ependymoma in one, and gliosis in one case. At median follow-up of 7 months, six (38%) patients experienced improved neurological function, eight (50%) remained stable, one (6%) experienced a delayed decrease in neurological function (GBM progression), and one (6%) died (GBM progression). Five (31%) patients developed persistent dysesthetic symptoms. Four (80%) patients with cystic tumors experienced neurological improvement compared to only two (18%) patients with noncystic tumors, p < 0.05. Preoperative steroid use (odds ratio, OR [95% confidence interval, CI] = 18.0 [1.24-260.1], p = 0.03) and cystic tumor (OR [95%CI] = 18.0 [1.24-260.1], p = 0.03) predicted neurological improvement after surgery. Radical resection of pediatric IMSCTs can be achieved with low incidence of neurological injury. Sensory syndromes frequently occur after pediatric IMSCT resection and frequently affect patient's quality of life. Tumors with compressive cysts may identify patients more likely to experience improved neurological function after surgical resection.

Use of Intraoperative Transcranial Motor Evoked Potentials to Predict Outcome after Resection of Pediatric Intramedullary Spinal Cord Tumors

Use of Intraoperative Transcranial Motor Evoked Potentials to Predict Outcome after Resection of Pediatric Intramedullary Spinal Cord Tumors

Intramedullary spinal cord tumors in children.

Pediatric intramedullary spinal cord tumors are rare and make up only a small percentage of all central nervous system neoplasms. These neoplasms are predominantly benign histologically, regardless of size, with a large percentage being astrocytomas or gangliogliomas. Ependymomas, which are common in adults, are relatively uncommon in children. Contemporary management of these tumors has generated much controversy. These neoplasms occur primarily in the cervical region, with pain or a motor deficit as the chief complaint. Surgery on these neoplasms can safely be performed using modern surgical adjuncts such as the ultrasonic aspirator, contact laser and neurophysiological monitoring. A radical resection of these tumors results in a good long-term outcome, since the majority are histologically benign. Adjuvant radiation therapy should only be administered for high-grade or malignant tumors. The majority of spinal cord tumors are benign; however, malignant tumors have a dismal outcome and surgery in these children should be limited to a conservative debulking. An attempt should be made for radical surgery in hope of avoiding radiation and chemotherapy for the benign tumors. Spinal deformity is a concern in these patients since 35% of children may require a stabilization procedure.

Pediatric intramedullary spinal cord tumors: special considerations.

Intramedullary spinal cord tumors (IMSCTs) of the pediatric population are rare and comprise thirty-five percent of intraspinal neoplasms. Low-grade astrocytomas predominate; ependymomas increase in frequency with ascending age and become the most frequent IMSCT in adults. Gangliogliomas are very rare in adults but comprise nearly thirty percent of tumors in children under three years of age. The cervical spine is the region of the spine most affected. Pain is the most common presenting symptom with weakness, gait deterioration, torticollis also frequently reported. Hydrocephalus occurs with greater frequency than in adult patients and often requires a shunt. Motor and sensory evoked potential monitoring is routinely utilized. Osteoplastic laminotomy is performed to forestall the development of progressive spinal deformity. Gross total resection is feasible in most ependymomas and results in surgical cure. Astrocytomas are infiltrating neoplasms and gross total resection is occasionally possible only in the pediatric population. However, the role of radical resection of low-grade fibrillary astrocytomas of the spinal cord in children has not been definitively demonstrated in the literature. Outcome for low-grade astrocytomas is better in children than adults, but not as favorable as that for ependymomas. Malignant tumors have dismal outcomes and surgery in these patients serves only to provide a diagnosis.

Pediatric intramedullary spinal cord tumors: is surgery alone enough?

Pediatric intramedullary spinal cord tumors occur infrequently with limited data on treatment strategies and outcome. Over an 11-year period, 44 patients with such tumors who were primarily managed by radical surgery were retrospectively reviewed. The majority of tumors (42/44, 95%) were in the cervical or thoracic spine and were histologically low grade. Gross total resection was achieved in 73% (32/44). At a median follow-up of 45 months, 11% (5/44) had severe weakness (modified McCormick scale IV-V), 66% (29/44) had mild-moderate deficits (I-III), and 16% (7/44) had sphincter dysfunction. Tumor recurrence rate was 18% (8/44) after a mean period of 54 months. Age, number of involved spinal cord segments and extent of surgical resection did not significantly impact on tumor recurrence rates (p = 0.28, 0.44 and 0.13, respectively). The only significant prognostic factor was histological type; mixed or higher grade tumors having a higher recurrence rate (p = 0.04). Radical surgery for intraspinal tumors can achieve long tumor-free survival without requiring adjuvant therapy.