Cord Tumors In Children Research Articles

Intraoperative Neurophysiologic Monitoring and Mapping During Surgery on Intramedullary Spinal Cord Tumors in Children and Adolescents.

Surgical resection of intramedullary spinal cord tumors carries significant risks of neurologic deficits, especially in cases of infiltrative tumors. In pediatric patients, this type of surgery may be associated with a high risk of poor neurologic outcome. Intraoperative neurophysiologic monitoring has been adopted as part of the clinical routine by many centers as a useful adjunct for intraoperative assessment of neurologic integrity. To what extent intraoperative neurophysiologic mapping strategies may further support intraoperative decision-making is still a matter of debate. Here, we report on a small cohort of five pediatric patients in whom mapping with the double-train paradigm was used to identify the dorsal column and corticospinal tract and to guide the surgical resection. We also discuss the possible benefits and challenges regarding the available literature.

Relevance of intraoperative motor evoked potentials and D-wave monitoring for the resection of intramedullary spinal cord tumors in children.

The purpose of this study was to evaluate the reliability of motor evoked potentials (MEP), somatosensory evoked potentials (SSEP), and D-wave monitoring as predictors of postoperative neurological deficits in pediatric patients undergoing resection of intramedullary spinal cord tumors (IMSCTs). Additionally, we aimed to determine whether alerts in the intraoperative neurophysiological monitoring (IONM) influenced the extent of resection (EOR). A retrospective analysis of the pediatric patients who underwent resection of IMSCT between March 2010 and April 2021 with an IONM guidance was performed. IONM alerts were recorded and correlated with patients' clinical status at discharge. Twenty three pediatric patients were included. MEP and SSEP were successfully elicited in all patients, while D-wave monitoring was feasible for 14 of them (60.9%). Significant IONM alerts occurred in 6 individuals (26.1%) with monitorable MEP and SSEP and 2 patients with monitorable D-waves (14.3%). The sensitivity, specificity, positive predictive value, and negative predictive value accounted for 100%, 81.8%, 20%, 100% for MEP, 100%, 92.3%, 50%, 100% for D-wave, and 50%, 81%, 20%, 94.44% for SSEP, respectively. Both MEP (p < 0.001) and D-wave monitoring (p < 0.001) accurately predicted postoperative motor deficits, while SSEP failed to provide significant accuracy regarding sensory deficits (p = 0.491). Gross-total tumor resection was performed in 29.4% of patients without IONM alerts and 33.3% of patients with IONM alerts, indicating that IONM alerts did not limit the EOR (p = 0.0857). MEP and D-wave monitoring can be perceived as reliable IONM modalities in pediatric IMSCTs surgery. Caution is needed with the implementation of SSEP to guide surgical decisions.

Intramedullary spinal cord tumors in children: the relevance of motor evoked potentials and D-wave monitoring

Intramedullary spinal cord tumors in children: the relevance of motor evoked potentials and D-wave monitoring

Особенности предиагностического периода клинических проявлений медуллобластом у детей в Республике Татарстан

The article provides up-to-date information on the first clinical symptoms of brain tumors in children and considers the term «pre-diagnostic symptomatic interval» of pediatric medulloblastoma, which is widely used in foreign pediatric and neuro-oncological practice. The authors publish the results of their own retrospective population study revealing the clinical and epidemiological characteristics of the most frequently detected malignant brain tumors in children — medulloblastomas in the pediatric population of the Republic of Tatarstan. The analysis of the pre-diagnostic symptomatic interval of this pathology was carried out through the prism of the LOWorPAY scheme, recommended by neurooncologists as a guide for early detection of tumors for primary care physicians. A probabilistic model of the clinical symptom complex is proposed, which determines the risk of neoplasm of the central nervous system in children. Its use will help increase the oncological alertness of doctors and systematize the tactics of detecting brain and spinal cord tumors in children on an outpatient basis.

Spinal cord tumors in children: A review of 21 cases treated at the same institution

IntroductionSpinal cord tumors in children (SCTC) are rare with a frequent diagnostic delay. Its management is multidisciplinary and challenging due to functional implications. The position of surgery is now better established but the role and timing of chemotherapy (CT) and radiotherapy (RT) still remains under debate. Adverse effects of treatments are important to be taken into account, in the follow-up of these children. The aim of this paper was to present a series of 21 cases of SCTC treated at the same institution, to briefly present clinical features, treatments and outcome, with a special focus on spinal deformities in children with this condition. Material and MethodsTwenty-one consecutive SCTC were referred to our institution from 1990 to 2014. Data regarding age, sex, diagnostic delay, clinical examination, MRI, surgery, pathology, other treatment (CT and RT), orthopedic issues and follow-up of these children were retrospectively recorded. ResultsMean age was 8years (standard deviation: 5.2years) (range: 4 months–17years). Mean diagnosis delay was 5.5 months (standard deviation: 6.5 months) (range: 0 days–18 months). All children (10 girls, 11 boys) were operated on (10 partial removals, 7 subtotal and 4 gross total removals) as first-line treatment. Pathological results showed 12 juvenile pilocytic astrocytomas, 1 grade III astrocytoma, 1 grade IV astrocytoma, 3 oligodendrogliomas, 2 ependymomas, 1 glioblastoma and 1 rhabdoid tumor. Fourteen children (66.7%) received additional treatment: 12 CT and 7 RT. Ten children had postoperative spinal deformities. Mean follow-up (FU) was 71 months (5 months–180 months), with a median FU at 60 months, where 8 tumor progressions and 4 deaths were observed. Overall, survival (at 5years) was 81% and progression free survival (at 5years) was 67%. ConclusionSurgery is the goal standard for SCTC and the only appropriate treatment in cases of a low-grade lesion with stable disease on MR follow-up. Additional treatment must be reserved for high-grade lesions or tumor progression not attainable by a second look surgery. Spinal deformities are a frequent complication. Overall, survival and event free survival primarily depends on the pathology. Studies involving more centers are obligatory with the aim of collecting more cases and drawing more definitive conclusions regarding the management of these tumors.

Treatment patterns of children with spine and spinal cord tumors: national outcomes and review of the literature.

Tumors of the spine in children are rare, and further clinical description is necessary. This study investigated epidemiology, interventions, and outcomes of pediatric patients with spine and spinal cord tumors. The National Inpatient Sample and Kids' Inpatient Database were used for the study. Outcomes were studied, and bivariate significant trends were analyzed in a multivariate setting. Analysis of 2870 patients between 2000 and 2009 found a median age of diagnosis of 11years (Tables 1 and 2). Most were white (65.2%) and had private insurance (62.3%), and 46.8% of procedures were emergent operations. Treatment occurred at teaching (93.6%) and non-children's hospitals (81.1%). Overall mortality rate was 1.7%, non-routine discharges occurred at a rate 19.9%, complications at 21.1%, and average total charges were $66,087. A majority of patients (87.5%) had no intervention, and of those patients receiving treatment, 78.2% underwent surgery and 23.1% had radiotherapy. Treatment with surgery alone increased significantly over time (p<0.0001). Odds ratio (OR) of mortality was significantly higher in 2006 (OR 3.5) and 2009 (OR 2.6) when compared to 2000. Complications (OR 7.9) and disease comorbidities (OR 1.5) were associated with significantly increased odds of mortality. Hospital characteristics, length of stay, and charges remained relatively unchanged. In recent years, there has been a decreasing incidence of spine and spinal cord tumors in children. Notably, a higher mortality rate is evident over time in addition to an increase in the proportion of patients undergoing surgery. The high percentage of emergent operations suggests a weak recognition of spine tumors in children and should prompt a call for increased awareness of this cancer. In spite of these findings, lack of tumor type identification was a limitation to this study.

Ostre poprzeczne zapalenie rdzenia kręgowego oraz guzy śródrdzeniowe u dzieci – obraz kliniczny, diagnostyka różnicowa, czynniki prognostyczne

Ostre poprzeczne zapalenie rdzenia kręgowego oraz guzy śródrdzeniowe u dzieci – obraz kliniczny, diagnostyka różnicowa, czynniki prognostyczne

Surgical management of intradural spinal cord tumors in children and young adults: A single-center experience with 50 patients.

Background:Intradural spinal cord tumors (IDSCTs) in children and young adults are rare diseases. This present study is aimed to demonstrate our experience with a large series of children and young adults with IDSCT.Methods:A total of 50 patients aged <20 years with IDSCT treated in our department between 1990 and 2010 were included in the study. Clinical, histological, and radiological findings, treatment strategies, and clinical outcome were retrospectively assessed. Depending on the relation to the spinal cord, IDSCT were dichotomized into intramedullary SCT (IMSCT) and extramedullary SCT (EMSCT). The functional outcome was evaluated with the Frankel score assessing the longest available follow-up period.Results:Mean age was 10.3 years (range 6 months–19 years). IDSCT surgery was performed in 44 patients (88%). A common first symptom in patients with EMSCT was neck and back pain (41%), whereas monoparesis of arms (43%) were often seen in patients with IMSCT. The main duration of the symptoms was longer in patients with IMSCT. The postoperative functional outcome was generally comparable to the preoperative functional condition, while better for EMSCT (P < 0.01). The functional outcome at last follow-up correlated significantly with the preoperative Frankel score (P < 0.002).Conclusion:Due to the mostly mild impact of the surgery on the functional outcome, the surgical treatment of IDSCT in children and young patients can be uniquely advocated.

Diffusion tensor imaging to guide surgical planning in intramedullary spinal cord tumors in children

Intramedullary spinal cord neoplasms (ISCN) in children provide diagnostic, treatment and management dilemmas. Resection results in the best chance for disease control, but the greatest risk of neurologic deficit. We hypothesize that diffusion tensor imaging (DTI) and diffusion tensor-fiber tracking (DT-FT) can help characterize margins of pediatric ISCN to aid in surgical planning. This HIPAA compliant retrospective study was performed after Institutional Review Board approval. Patients with ISCN from a single tertiary care pediatric institution were identified, and patients with preoperative DTI were evaluated. Ten patients (eight males and two females) with ISCN with preoperative DTI were identified. The mean age was 11.1 ± 6.2 years (range, 2-18 years). Eight tumors demonstrated DTI and DT-FT evidence of splayed cord tracts, and two demonstrated evidence of infiltration of cord tracts. The eight patients with splayed tracts underwent resection, with seven achieving gross-total resection and one subtotal resection. The two patients with infiltration of white matter tracts underwent biopsy of their lesion. DTI of pediatric ISCN can aid in defining the margins of the tumor and relationship to the intrinsic white matter structures of the spinal cord. Splaying and displacement of fiber tracts appears to predict a discrete margin to the tumor and resectability, whereas infiltration of the white matter tracts suggests biopsy may be more advisable.

Quality of life after surgical treatment of primary intramedullary spinal cord tumors in children

Presently, the best available treatment for intramedullary spinal cord tumors (IMSCTs) in children is microsurgery with the objective of maximal tumor removal and minimal neurological morbidity. The latter has become manageable with the development and standard use of intraoperative neurophysiological monitoring. Traditionally, the perioperative neurological evaluation is based on surgical or spinal cord injury scores focusing on sensorimotor function. Little is known about the quality of life after such operations; therefore, this study was designed to investigate the impact of surgery for IMSCTs on the quality of life in children. Twelve consecutive pediatric patients treated for IMSCT were included in this retrospective fixed cohort study. A multidimensional questionnaire-based quality of life instrument, the Pediatric Quality of Life Questionnaire version 4 (PedsQL 4.0), was chosen to analyze follow-up data. This validated instrument particularly allows for a comparison between a patient cohort and a healthy pediatric sample population. Of 11 mailed questionnaires (1 patient had died of progressive disease), 10 were returned, resulting in a response rate of 91%. There were 8 low-grade lesions (5 pilocytic astrocytomas, 1 ganglioglioma, 1 hemangioblastoma, and 1 cavernoma) and 4 high-grade lesions (2 anaplastic gangliogliomas, 1 glioblastoma, and 1 glioneuronal tumor). The mean age at diagnosis was 7.5 years, the mean follow-up was 4.2 years, and 83% of the patients were male. Total resection was achieved in 5 patients and subtotal resection in 7. Four patients had undergone 2 or more resections. The 4 patients with high-grade tumors and 2 with incompletely resected low-grade tumors underwent adjuvant treatment (2 chemotherapy and 4 both radiotherapy and chemotherapy). The mean modified McCormick Scale score at the time of diagnosis was 1.7; at the time of follow-up, 1.5. The mean PedsQL 4.0 total score in the low-grade group was 78.5; in the high-grade group, 82.6. There was no significant difference in PedsQL 4.0 scores between the patient cohort and the normal population. In a small cohort of children who had undergone surgery for IMSCTs with a mean follow-up of 4.2 years, quality of life scores according to the PedsQL 4.0 instrument were not different from those in a normal sample population.

Torticollis as a first sign of posterior fossa and cervical spinal cord tumors in children

Torticollis, despite being well-known neurological manifestation, is often underestimated as a first symptom of the abnormalities of posterior cranial cavity and cervical spinal cord. The purpose of this study is to analyze the occurrence of acquired torticollis in children as a herald sign of the tumors of the cervical spinal cord or of the posterior fossa. Clinical records of 54 cases treated for the tumor of the cervical spinal cord or posterior fossa (including congenital ones) were retrospectively reviewed. The following data were calculated: the occurrence of the torticollis as a first sign of tumors, the duration time from the onset of the symptoms to diagnosis, the concurrence of other pathological symptoms, and the diminishing of symptoms of the torticollis following treatment. In 12/54 (22.2 %) torticollis was first sign of central nervous system tumor and in all of them preceded other neurological symptoms. The time from the onset of torticollis to establishing diagnosis ranged from 2 to 52 weeks (9.6 weeks on average). Eleven of twelve patients were treated surgically--in 10 of them, torticollis disappeared in the postoperative course. Torticollis may be a herald sign of the tumor of the cervical spinal cord or the posterior fossa. Those pathologies should be considered in the differential diagnosis of the torticollis, particularly if accompanied by other symptoms of the focal pathology of central nervous system. Awareness of this fact may shorten the time to establish the proper diagnosis. Torticollis necessitates exclusion of the posterior fossa and spinal cord tumor.

Spinal cord tumors in children under the age of 3 years: a retrospective Canadian review

Tumors of the spinal cord are exceedingly rare in infancy and only a paucity of literature exists describing the spectrum of this disease and its management. The objectives of our study were to describe the demographic characteristics of spinal cord tumors (SCT) in children less than 3 years of age at diagnosis and to review their treatment and outcome. A national retrospective chart review was conducted on patients under the age of 3 years diagnosed with a primary tumor of the central nervous system (CNS) between 1990 and 2005 across Canada. Inclusion criteria were: age ≤ 3 years, histologic confirmation of the diagnosis, and residency in Canada. A centralized database was created and information regarding SCT was extracted. Twenty-five of five hundred seventy-nine patients (4.3%) in the data bank had a SCT. The majority of tumors were low-grade astrocytomas (14/25). Leptomeningeal dissemination based on neuroradiologic imaging and/or cerebrospinal fluid cytology was present in five (20%) patients. The majority of patients underwent an incomplete surgical resection (52%). Most patients (64%) did not receive postoperative radiotherapy or chemotherapy. Seventy-two percent (18/25) developed recurrent/progression of disease. Overall 2- and 5-year survival for low- and high-grade malignancies was 93 ± 6.4% and 37.5 ± 17.1% respectively. Significant predictors of survival included mean duration of symptoms prior to initial diagnosis and recurrence/progression of disease. Relapse/progression of disease in infant SCT is frequent. Prolonged survival of low-grade tumors is possible with further therapy; however, the prognosis of high-grade malignancies remains poor.

Incidence of spinal deformity after resection of intramedullary spinal cord tumors in children who underwent laminectomy compared with laminoplasty

Incidence of spinal deformity after resection of intramedullary spinal cord tumors in children who underwent laminectomy compared with laminoplasty

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.

Incidence of spinal deformity after resection of intramedullary spinal cord tumors in children who underwent laminectomy compared with laminoplasty

Gross-total resection of pediatric intramedullary spinal cord tumor (IMSCT) can be achieved in the majority of cases while preserving long-term neurological function. Nevertheless, postoperative progressive spinal deformity often complicates functional outcome years after surgery. The authors set out to determine whether laminoplasty in comparison with laminectomy has reduced the incidence of subsequent spinal deformity requiring fusion after IMSCT resection at their institution. The first 144 consecutive patients undergoing resection of IMSCTs at a single institution underwent laminectomy with preservation of facet joints. The next 20 consecutive patients presenting for resection of IMSCTs underwent osteoplastic laminotomy regardless of patient or tumor characteristics. All patients were followed up with telephone interviews corroborated by medical records for the following outcomes: 1) neurological and functional status (modified McCormick Scale [MMS] score and Karnofsky Performance Scale [KPS] score); and 2) development of progressive spinal deformity requiring fusion. The incidence of progressive spinal deformity and the long-term neurological function were compared between the laminectomy and osteoplastic laminotomy cohorts. The means are expressed +/- the standard deviation. Overall, the patients' mean age was 8.6 +/- 5 years, and they presented with median MMS scores of 2 (interquartile range [IQR] 2-4). A > 95% resection was achieved in 125 cases (76%). There were no differences (p > 0.10) between patients treated with osteoplastic laminotomy and those treated with laminectomy in terms of the following characteristics: age; sex; duration of symptoms; location of tumor; incidence of preoperative scoliosis (Cobb angle > 10 degrees : 7 [35%] with laminoplasty compared with 49 [34%] with laminectomy); involvement of the cervicothoracic junction (7 [35%] compared with 57 [40%]); thoracolumbar junction (4 [20%] compared with 36 [25%]); tumor size; extent of resection; radiation therapy; histopathological findings; or mean operative spinal levels (7.5 +/- 2 compared with 7.5 +/- 3). Nevertheless, patients who underwent osteoplastic laminotomy had better median preoperative MMS scores than those treated with laminectomy (2 [IQR 2-2] compared with 2 [IQR 2-4]; p = 0.04). A median of 3.5 years (IQR 1-7 years) after surgery, only 1 patient (5%) in the osteoplastic laminotomy cohort required fusion for progressive spinal deformity, compared with 43 (30%) in the laminectomy cohort (p = 0.027). Adjusting for the inter-cohort difference in preoperative MMS scores, osteoplastic laminotomy was associated with a 7-fold reduction in the odds of subsequent fusion for progressive spinal deformity (odds ratio 0.13, 95% confidence interval 0.02-1.00; p = 0.05). The median MMS and KPS scores were similar between patients who underwent osteoplastic laminotomy and those in whom laminectomy was performed (MMS Score 2 [IQR 2-3] for laminotomy compared with 2 [IQR 2-4] for laminectomy, p = 0.54; KPS Score 90 [IQR 70-100] for laminotomy compared with 90 [IQR 80-90] for laminectomy, p = 0.545) at a median of 3.5 years after surgery. In the authors' experience, osteoplastic laminotomy for the resection of IMSCT in children was associated with a decreased incidence of progressive spinal deformity requiring fusion but did not affect long-term functional outcome. Laminoplasty used for pediatric IMSCT resection may decrease the incidence of progressive spinal deformity requiring subsequent spinal stabilization in some patients.

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 after resection of cervical intramedullary spinal cord tumors in children

Progressive spinal deformity after cervical intramedullary spinal cord tumor (IMSCT) resection requiring subsequent fusion occurs in many cases among pediatric patients. It remains unknown which subgroups of patients represent the greatest risk for progressive spinal deformity. The data for 58 patients undergoing surgical resection of cervical IMSCT at a single institution were retrospectively collected and analyzed for development of progressive spinal deformity requiring fusion. The association of all clinical, radiographic, and operative variables to subsequent progressive spinal deformity as a function of time was assessed via Kaplan-Meier plots and Log-rank and Cox analyses. Mean age at the time of surgery was 11 +/- 6 years. Eleven (19%) patients required subsequent fusion for progressive spinal deformity at a median [interquartile range (IQR)] of 4 (2-6) years after IMSCT resection. Five (36%) of 14 patients with preoperative scoliosis or loss of lordosis developed postoperative progressive spinal deformity compared to only 6 (13%) of 44 patients with normal preoperative sagittal and coronal balance, p = 0.06. Patients <13 years of age were more than three times more likely to develop postoperative progressive deformity, p = 0.05. Decompression spanning both the axial cervical spine (C1-C2) and the cervico-thoracic junction (C7-T1) increased the risk for progressive spinal deformity fourfold, p = 0.04. Number of spinal levels decompressed, revision surgery, radiotherapy, involvement of C1-C2 or C7-T1 alone in the decompression, or any other recorded variables were not associated with progressive postoperative spinal deformity. Patients possessing one or more of these characteristics should be monitored closely for progressive spinal deformity after surgery.

Childhood Spinal Glioblastoma Multiforme with Intracranial Dissemination

Primary spinal cord tumors in children are uncommon and account for approximately 5% of all pediatric central nervous system tumors. Intracranial metastases from primary spinal cord tumors have rarely been reported. Spinal cord glioblastoma in children mainly involves the thoracic region, whereas involvement of the medullaris conus with intracranial dissemination has been described in six cases. This paper describes a pediatric case of thoracic glioblastoma multiforme with intracranial dissemination in the early stage of the clinical course and discuss the clinical and neuroradiological manifestations, the possible patterns of dissemination and finally to consider the therapeutic implications.

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.