Intramedullary spinal cord tumors in pediatric patients presenting later with brain lesions: case series and systematic review of the literature.

PURPOSE: Intramedullary spinal cord tumors are an uncommon pathology in adults and children. Most descriptive studies of intramedullary spinal cord tumors have not focused on a possible association with future brain lesions. To the best of our knowledge, few reports describe this potential relationship. This is one of the most extensive case series of secondary brain lesions of intramedullary spinal cord tumors in the pediatric population. METHODS: Retrospective chart review was performed on pediatric patients (21 years old and younger) who underwent resection of an intramedullary spinal cord tumor at two tertiary care hospitals from 2001 to 2020. Patients previously treated or diagnosed with spinal cord tumor, and subsequent development of intracranial manifestation of the same or different tumor, were included. Data regarding epidemiology, surgical intervention, and clinical and follow-up course were gathered. Data analysis was performed according to a standardized clinical protocol with a literature review. RESULT: More than 500 patients underwent intradural spinal tumor resection surgeries at participating hospitals from 2001 to 2020. After excluding adult patients (older than 21 years old) and those with extramedullary lesions, 103 pediatric patients were identified who underwent resection of an intramedullary spinal cord tumor. Four underwent resection of an intermedullary tumor and later in their follow-up course developed a secondary intracranial neoplasm. In every case, the secondary neoplasm had the same pathology as the intramedullary tumor. Three of the patients had tumors at the cervico-thoracic junction, and one patient had a high cervical tumor. These patients had a negative primary workup for any metastatic disease at the time of the presentation or diagnosis. Complete and near complete resection was performed in three patients and subtotal in one patient. CONCLUSION: Secondary brain tumors disseminated after initial spinal cord tumor are extremely rare. This study aims to allow specialists to better understand these pathologies and treat these rare tumors with more certainty and better expectations of unusual associated lesions and conditions.

Epidemiological features of spinal intradural tumors, a single-center clinical study in Beijing, China.

BACKGROUND: Spinal intradural tumors are rare and heterogeneous in histological type, aggressiveness, and symptomatology, and there is a lack of data about them. This study investigated the epidemiological features of spinal intradural tumors. METHODS: This retrospective analysis included patients with spinal intradural tumors who underwent surgical treatment at the Myelopathy and Spondylosis Ward Beijing Jishuitan Hospital between January 2012 and December 2022. RESULTS: This study included 1321 patients [aged 47.19 ± 14.90 years, 603 (45.65%) males] with spinal intradural tumors. The most common histological subtype was schwannoma [n = 511 (38.68%)], followed by spinal meningioma [n = 184 (13.93%)] and ependymoma [n = 101 (7.65%)]. Fifteen (1.14%) patients were diagnosed with metastatic spinal intradural tumors as a presentation of another primary cancer type. The spinal intradural tumors were mostly found in the lumbar region [n = 436 (33.01%)], followed by the thoracic vertebrae [n = 390 (29.52%)], cervical vertebrae [n = 154 (11.66%)], and thoracolumbar region [n = 111 (8.40%)]. Schwannomas mostly affected the lumbar region [n = 256 (52.64%)], spinal meningiomas in the thoracic region [n = 153 (83.15)], and ependymomas in the lumbar region [56 (55.45%)]. The de novo metastases were mostly found in the lumbar region [n = 8 (53.33%)]. CONCLUSION: According to the results of our single-center study, the most common spinal intradural tumor in Northern China is schwannoma, followed by spinal meningioma and ependymoma.

A current review of spinal meningiomas: epidemiology, clinical presentation and management.

PURPOSE: To provide an up-to-date review of the epidemiology, histopathology, molecular biology, and etiology of spinal meningiomas, as well as discuss the clinical presentation, clinical evaluation, and most recent treatment recommendations for these lesions. METHODS: PubMed and Google Scholar search was performed for studies related to meningiomas of the spine. The terms "meningioma," "spinal meningioma," "spine meningioma," "meningioma of the spine," "benign spinal tumors," and "benign spine tumors," were used to identify relevant studies. All studies, including primary data papers, meta-analyses, systematic reviews, general reviews, case reports, and clinical trials were considered for review. RESULTS: Eighty-four studies were identified in the review. There were 22 studies discussing adverse postoperative outcomes, 21 studies discussing tumor genetics, 19 studies discussing epidemiology and current literature, 9 studies discussing radiation modalities and impact on subsequent tumor development, 5 studies on characteristic imaging findings, 5 studies discussing hormone use/receptor status on tumor development, 2 discussing operative techniques and 1 discussing tumor identification. CONCLUSION: Investigations into spinal meningiomas generally lag behind that of intracranial meningiomas. Recent advancements in the molecular profiling of spinal meningiomas has expanded our understanding of these tumors, increasing our appreciation for their heterogeneity. Continued investigation into the defining characteristics of different spinal meningiomas will aid in treatment planning and prognostication.

Brain and Spinal Cord Tumors Among the Life-Threatening Health Problems: An Introduction.

As one of the global concerns, cancers, including brain and spinal cord tumors, are responsible for mortalities and irreversible morbidities in the affected patients. Although advancements in molecular pathology and imaging of tumors may have influenced the incidence rate due to higher diagnosis in early stages, exposure to environmental risk factors could be another explanation for increased incidence of these tumors over the past decades. Similar to many other tumors, the CNS tumors begin in cellular dimension with activation of different molecular pathways. Several genetic, epigenetic, and immunologic pathways and processes are already discovered to play roles in pathophysiology of these tumors, which mostly will eventually become symptomatic. Each of these tumors may exhibit imaging characteristics, making it possible to list a series of differential diagnosis before histopathologic examination. Advances in molecular pathology have resulted in better understanding and categorization of CNS tumors, leading to better decision-making on the most appropriate therapeutic approach for each category, as well as proposing new therapeutic modalities to treat these tumors. As an introduction to the 2-volume book, this chapter addressed different types of human brain and spinal cord tumors based on the fifth version of WHO classification of CNS tumors.

The Epidemiology of Brain and Spinal Cord Tumors.

CNS tumors are a diverse group of neoplasms that emerge from a variety of different CNS cell types. These tumors may be benign, malignant, or borderline in nature. The majority of high grade glial tumors are fatal, with the exception of pilocytic astrocytoma. Primary malignant CNS tumors occur at a global annual rate of 2.1 to 5.8 per 100,000 persons. Males are more likely to develop malignant brain tumors than females, whereas benign meningiomas are more common in adult females. Additionally, gender inequalities in non-malignant tumors peak between the ages of 25 and 29 years. Only a small number of genetic variants have been associated with survival and prognosis. Notably, central nervous system (CNS) tumors exhibit significant age, gender, and race variation. Race is another factor that affects the incidence of brain and spinal cord tumors. Different races exhibit variation in terms of the prevalence of brain and CNS malignancies. This chapter discusses ongoing research on brain and spinal cord tumor epidemiology, as well as the associated risks and accompanied disorders.

The impact of body mass index and height on risk for primary tumours of the spinal cord, spinal meninges, spinal and peripheral nerves in 1.7 million norwegian women and men: a prospective cohort study.

BACKGROUND: Primary tumours of the spinal cord, spinal meninges, spinal and peripheral nerves comprise a heterogenous group of pathology, dominantly represented by meningioma, nerve sheath tumours (NST) and glioma. Body height and body mass index (BMI) are risk factors for certain brain tumour subgroups, but no other study has specifically assessed height and BMI in relation to primary tumours of the spine and peripheral nerves in women and men. METHODS: In this prospective population-based cohort study height and weight were measured in 1.7 million adult Norwegian women and men at baseline. Incident cases of primary tumours arising from the spinal cord, spinal meninges, spinal and peripheral nerves during follow-up were identified by linkage to the National Cancer Registry. Tumour risk was assessed by Cox regression analyses in relation to height and BMI. RESULTS: During 49 million person-years of follow-up, 857 primary tumours of the spinal cord, spinal meninges, spinal and peripheral nerves were identified. Overweight and obesity were not associated with risk for all tumours or any tumour subgroup. Height was positively associated with risk for all tumours (HR per 10 cm increase: 1.30, 95% CI 1.16-1.46). The association between height and tumour risk varied between tumour subgroups: while height was not significantly associated with NST, height increased the risk for meningioma (HR 1.42, 95% CI 1.13-1.78) and glioma (HR 1.56, 95% CI 1.06-2.28). The strongest association between height and tumour risk was found for the glioma subgroup of ependymoma in women (HR 3.38, 95% CI 1.64-6.94). CONCLUSION: This study could not identify overweight and obesity as risk factors for primary tumours of the spinal cord, spinal meninges, spinal and peripheral nerves in women or men. Increasing body height was associated with increased tumour risk overall, but not universal for all tumour subgroups.Importance of the studyPrimary tumours of the spinal cord, spinal meninges, spinal and peripheral nerves have received little focus in epidemiologic studies, although the incidence and histo-pathological tumour subgroups differ significantly from primary brain tumours. Risk factors for these tumours have hardly been assessed in previous studies. Height, overweight and obesity are known risk factors for several cancers, including certain brain tumour subgroups, such as meningioma.This is the first study to report the association between height, overweight and obesity and primary tumours of the spinal cord, spinal meninges, spinal and peripheral nerves. This includes tumour subgroups of meningioma, nerve sheath tumour, glioma and the most common spinal glioma subgroup of ependymoma. While overweight and obesity were not associated with either of the tumour subgroups, an association between increasing body height and risk for spinal meningioma and glioma, including ependymoma, was found. Nerve sheath tumour risk was not associated with increasing body height.

Anaplastic gangliogliomas of the spinal cord: a scoping review of the literature.

Gangliogliomas (GGs) are rare, usually low-grade tumors that account for 1-2% of all central nervous system (CNS) neoplasms. Spinal GGs are exceedingly rare (1% of all spinal tumors) and the presentation of anaplastic features in them is even rarer. According to the last World Health Organization (WHO) classification of CNS neoplasms, anaplastic GG (AGG) is classified as a malignant neoplasm (grade III). We performed a scoping review of the literature to elucidate the epidemiology, clinical features, histopathology, treatment, and outcome of primary spinal AGGs, which, to the best of our knowledge, is the first such review. Relevant studies were identified by a search of the MEDLINE and SCOPUS databases, using the following combination of search strings: (anaplastic ganglioglioma or malignant ganglioglioma or high grade ganglioglioma) AND (spine or spinal or spinal cord). We included studies related to primary or recurrent AGGs and malignant transformation of low-grade GGs. The search produced 15 eligible studies, plus two studies from the references, all of which were case reports of patients with spinal AGGs (17 studies with 22 patients). The mean age of the patients was 21.4 years and the sex ratio was 1:1, with male predominance. Motor impairment was the most common presentation, followed by sensory impairment, gait problems, urinary disturbances, and back pain. The thoracic spine was the most frequently involved area (14/22) followed by the cervical (6/22) and lumbar (5/22) spine. In terms of histology, the anaplastic features were usually predominant in the glial element, resembling high-grade astrocytomas, while the neuronal element was composed of the so-called dysplastic ganglion (neuronal) synaptophysin-positive cells, without mitotic figures. Complete surgical resection of the tumor without neurological compromise, plus adjuvant chemotherapy and radiotherapy, was the treatment protocol implemented in the two patients with the best outcome. Primary spinal AGG is an exceedingly rare entity, with only 22 cases being retrieved after an extensive literature search. They appear to affect children and young adults and tend to manifest aggressive behavior. Most studies report that only the glial component of AGGs presents high-grade malignant features, with low mitotic activity in the neuronal component. We therefore suggest that, pending novel targeted therapy, AGGs should be treated as high-grade gliomas, with an aggressive treatment protocol consisting of maximal safe resection and adjuvant chemotherapy and radiotherapy.

Paediatric Spinal Tumours: Profile and Treatment Outcome in a Nigerian Tertiary Institution.

INTRODUCTION: Paediatric spinal tumours are rare, accounting for 1-10% of all childhood central nervous system tumours. There is a paucity of information on spinal tumours in Sub-Saharan Africa. This is particularly so in the Nigerian paediatric population where neuro-oncologic data are limited. Indeed, there is no previously published work on paediatric spinal tumours in the Nigerian neurosurgical literature. The authors aim to document the profile of paediatric spinal tumours in a Nigerian tertiary institution and to contribute to the available data on paediatric central nervous system tumours in West Africa. METHODS: We retrospectively evaluated data on paediatric patients who underwent surgery for spine tumours over a 20-year period at our institution. RESULTS: A total of 12 patients were managed for paediatric spine tumours during the study period. These had a male:female ratio of 1:1.4, and their ages ranged between 3 and 18 years (mean: 12.83 ± 4.75 years). The highest incidence (6/12) was seen in the 15-18 years age group. The mean duration of symptoms was 10.2 months. More than half (7/12) of the patients presented with symptoms with duration of at least 6 months. Motor deficit was present in all patients at presentation. Two-thirds of the tumours were in the thoracic region, 1 was located in the cervical region, while the remaining 3 tumours were cervicothoracic. The tumour was extradural in location in 8 of our patients and intramedullary in the remaining 4. Astrocytoma, intramedullary in all cases, was the most predominant histological tumour type (3/12) in our series. Postoperative neurological improvement occurred in 7 of the patients while 5 remained the same. There was no permanent postoperative neurological deterioration or perioperative mortality. CONCLUSION: Paediatric spinal tumours mostly affected older children in our study group and were predominantly astrocytic in nature. The most common tumour location was extradural, involving mostly the thoracic spinal level. The preoperative neurological status correlates with the postoperative functional outcome. Therefore, the need for early diagnosis and treatment of these tumours cannot be overemphasized.

Hydrocephalus in primary intradural spinal cord tumors: a systematic review of the literature in the pediatric population.

Hydrocephalus in children with primary intradural spinal cord tumors is exceedingly rare. Herewith, we performed a systematic literature review to address epidemiology, suggested pathophysiological mechanisms, prognostic factors, and treatment of such cases. We performed a systematic review with the best available evidence on cases of pediatric primary intradural tumors of the spinal cord presented with hydrocephalus. The patients were subjected to quantitative analysis on a basis of epidemiological features (age, sex, tumor type and location, clinical presentation, survival, dissemination). The possible pathophysiological theories are discussed in detail. Forty-four studies with a total of 121 patients were included in the study. Astrocytomas were the most frequent tumor (64.5%) type, while most tumors were located in cervical (31.4%) or cervicothoracic region (25.6%). About half of the cases concerned children under 6 years of age. The block of subarachnoid CSF (cerebrospinal fluid) pathways from disseminated tumor cells and the neoplastic inflammation caused by tumor elements advocated to be the major pathogenetic mechanisms. Surgical excision of the tumor and hydrocephalus treatment is usually performed. Primary intradural spinal cord tumors should be considered in children with communicative hydrocephalus of unknown etiology. Onset of hydrocephalus after tumor removal is related to higher mortality.

Primary intraspinal benign tumors treated surgically: an analysis from China.

PURPOSE: The purpose of this study was to review the clinical features in a cohort of Chinese patients with primary intraspinal benign tumors. METHODS: This retrospective study included a consecutive series of patients with intraspinal benign lesions who received surgery between January 2014 and October 2018 at our hospital. We collected each patient's clinical data, including age, gender, presenting symptoms, the spinal level (cervical, thoracic, lumbar, or sacral), and location (intramedullary or extramedullary) of the tumor. RESULTS: A total of 66 patients were included in this study, of whom 24 were men and 42 (63.6%) were women. The mean age was 52.5 years (range, 21-76 years). The most common symptom was sensory deficits. The most common tumor type, found in 56.1% patients, was schwannoma, followed by meningioma in 33.3% patients. The commonly performed surgery included decompression of spinal canal and excision of spinal lesions. CONCLUSION: Primary intraspinal benign tumors occur in elderly and female population and at the thoracic region. Schwannoma and meningioma are the two with higher incidence. The surgical outcome in terms of tumor excision and functional recovery is good.

[Spinal Cord Tumors:Classification, Treatment, and Prognosis].

Spinal cord tumors are classified into three types according to the site of origin: epidural tumors, intradural extramedullary tumors, and intramedullary tumors. It is important to understand the common tumors at each site. Most intradural extramedullary tumors are benign schwannomas or meningiomas and need surgical removal. For intramedullary tumors, it is important to determine the type of tumor and the site of origin before selecting the surgical approach.

Intramedullary spinal cord metastases: an institutional review of survival and outcomes.

INTRODUCTION: Intramedullary spinal cord metastases (ISCMs) are rare lesions affecting the spinal cord in patients with disseminated malignancy. Today, due to increased survival, these lesions are more frequently diagnosed. Literature on survival and neurologic outcomes is sparse. Herein, we describe a single institutional case series on ISCMs reported to date in the English literature. METHODS: We retrospectively analyzed the medical records of patients diagnosed with intramedullary metastatic lesions at our institution between 1997 and 2016. We analyzed different approaches to management and factors influencing survival and neurologic outcomes. RESULTS: A total of 70 patients (86 lesions) were analyzed. Most lesions were found in thoracic spinal cord (50%) followed by cervical (34%) and conus medullaris (14%). Mean age at diagnosis was 55.6 ± 10.6 years with 60% (n = 42) being females. Median survival was 104.5 days (range 1-888 days). Twenty-three patients (33%) received conservative management, 39 (56%) received palliative radiotherapy, whereas 8 (11%) underwent surgery with one patient receiving only a biopsy. Age, sex, presence of concomitant brain and other systemic metastasis didn't influence survival. Patients with solitary metastases had longer survival compared to multiple lesions (3.6 vs. 2.2 months, p = 0.01). In patients with solitary lesions without brain metastasis, surgical resection was associated with significantly longer survival (6 months vs. 3 months, p = 0.02). CONCLUSION: The overall survival in patients with intramedullary metastasis remains poor. Surgical management may contribute to improved survival and neurologic outcomes in selected patients. Intramedullary metastasis may have a greater role on overall survival compared to systemic metastatic burden.

Variation in management of spinal gliobastoma multiforme: results from a national cancer registry.

PURPOSE: Primary glioblastoma of the spinal cord (spinal GBM) is a rare central nervous system tumor, relative to its cranial counterpart (cranial GBM). Our current knowledge of spinal GBM epidemiology, tumor characteristics and treatment are insufficient and mostly based on single-institution case series. METHODS: All patients diagnosed with grade-4 GBM from 2004 to 2014 were queried from the National Cancer Database. Chi square analysis was used to compare presenting characteristics while Kaplan-Meier and Cox regression analyses were employed for survival analyses. RESULTS: Total 103,496 patients with cranial GBM and 190 patients with spinal GBM were analyzed. Median survival for spinal GBM was found to be higher compared to cranial GBM (p = 0.07). Spinal GBM patients had significant better survival in 18 to 65 years age group than < 18 years and > 65 years age group (p = 0.003). Overall survival time for 95 spinal GBM patients with available treatment data was not statistically different among the four treatment modalities (radiation with or without chemotherapy, surgery alone, surgery with adjuvant therapy, and palliative therapy; p = 0.28).On multivariable analysis, < 18 years age group was associated with improved survival (HR 0.50, 95% CI 0.23-1.00, p = 0.046), while tumor extension was associated with poor survival (HR 2.71, 95% CI 1.04-6.22, p = 0.041). Interestingly surgery with adjuvant therapy was unable to show increase survival compared to other treatment modalities. CONCLUSIONS: Our study adds to the growing literature on spinal GBM with a focus on comparative trends with cranial GBM and outcomes with different treatment modalities.

Primary brain and other central nervous system tumors in Appalachia: regional differences in incidence, mortality, and survival.

BACKGROUND: The Appalachian region is a large geographic and economic area, representing 7.69% of the United States (US). This region is more rural, whiter, older, and has a higher level of poverty as compared to the rest of the US. Limited research has been done on primary brain and other central nervous system tumors (PBT) epidemiology in this region. In this analysis we characterize incidence, mortality, and survival patterns. METHODS: Data from 2006 to 2015 were obtained from the central brain tumor registry of the US (provided by CDC and NCI). Appalachian counties were categorized using the Appalachia Regional Council scheme. Overall and histology-specific age-adjusted incidence and mortality rates per 100,000 population were generated. 1-, 5-, and 10-year relative survival (RS) was estimated using CDC national program of cancer registry data from 2001 to 2014. RESULTS: Overall PBT incidence within Appalachia was 22.62 per 100,000, which is not significantly different from the non-Appalachian US (22.77/100,000, p = 0.1189). Malignant incidence was 5% higher in Appalachia (7.55/100,000 vs. 7.23/100,000, p < 0.0001), while non-malignant incidence was 3% lower (15.07/100,000 vs. 15.54/100,000, p < 0.0001). 5-year RS for malignant PBT was lower (31.4% vs. 36.0%), and mortality due to malignant PBT was higher in Appalachia (4.86/100,000 vs. 4.34/100,000, p < 0.0001). CONCLUSION: Appalachia has increased malignant and decreased non-malignant PBT incidence, and poorer survival outcomes for malignant PBT compared to the non-Appalachian US.

Symptomatic postoperative spinal epidural hematoma after spine tumor surgery: Incidence, clinical features, and risk factors.

STUDY DESIGN: Case-control study. OBJECTIVES: The objective of this study was to provide some useful information concerning the incidence, clinical features, and risk factors for symptomatic postoperative spinal epidural hematoma (SPSEH) in an isolated cohort of patients undergoing spine tumor surgery. SETTING: Hospital in Shanghai, China. METHODS: We retrospectively reviewed all patients who underwent surgery for spine tumors between August 2012 and August 2017, and conducted a case-control study involving 16 patients who received evacuation surgery due to SPSEH after spine tumor surgery and 48 controls without SPSEH. Case and control subjects were matched at 1:3 by pathological diagnosis, tumor size (±1 cm), resection mode, surgical approach, and the operation team. Data of SPSEH subjects along with 48 matched controls were further obtained from a detailed review of the medical records. Univariate and multivariate analyses were conducted to identify the risk factors for developing SPSEH. RESULTS: SPSEH evacuation surgery was performed after 16 of 5421 (0.30%) spine tumor surgeries. Angiogenic tumors were the most susceptible tumors developing SPSEH. Very large hematomas, continuous blood loss, and delayed hematomas were characteristic clinical presentations for SPSEH after spine tumor surgery. Multiple logistic regression analysis suggested that patients suffering from at least one medical comorbidity and patients with Frankel grade of A-C had a significantly higher risk of developing SPSEH. CONCLUSIONS: The incidence of SPSEH after spine tumor surgery requiring surgical evacuation was 0.30%. Medical comorbidity and Frankel grade were identified as independent risk factors for SPSEH development.

Changes in sagittal alignment after surgical excision of thoracic spinal cord tumors in adults.

STUDY DESIGN: Retrospective chart audit. OBJECTIVES: This study investigated changes in sagittal alignment in adults after excision of thoracic spinal cord tumors without spinal fixation. SETTING: Single-center study at an academic orthopedic department in Japan. METHODS: We retrospectively reviewed records for 32 adults who underwent excision of thoracic spinal cord tumors by multilevel laminectomies without fixation. The participants were divided according to whether the tumor was in the upper (T1-4), middle (T5-8), or lower (T9-12) thoracic spine. We analyzed parameters such as age, sex, time in surgery and estimated blood loss, follow-up period, and preoperative and follow-up the Japanese Orthopaedic Association (JOA) scores and radiographs. RESULTS: Postoperative T1-12 kyphotic changes did not correlate with age, the number of resected laminae, or preoperative T1-12 kyphosis. JOA recovery rates were similar regardless of the tumor location. Participants with tumors in the upper thoracic spine had significant postoperative increases in T1-4 kyphosis, T1 slope (p < .05, respectively). In contrast, there were no significant changes in alignment in participants with tumors in the middle or lower thoracic spine. CONCLUSION: Even without fixation, sagittal alignment did not change after surgery to excise tumors in the middle and lower thoracic spine, indicating that fixation may not be necessary when excising spinal cord tumors in this region. In contrast, postoperative kyphosis may increase when the tumor is in the upper thoracic spine.

Maternal Folate Intake and Risk of Childhood Brain and Spinal Cord Tumors: A Systematic Review and Meta-Analysis.

BACKGROUND: Many epidemiological studies have investigated the effect of maternal diet and prenatal multivitamin supplementation on pediatric cancer risk. Childhood brain and spinal cord tumors (CBSCT) have been attributed to different possible risk factors. METHODS: We conducted a systematic review and meta-analysis on maternal folate intake before and during pregnancy and the risk of CBSCT. We systematically reviewed publications obtained by searching the Insitute for Scientific Information Web of Knowledge and PubMed literature databases. We extracted the risk estimate of the highest and the lowest reported categories of intake from each study and conducted a meta-analysis using a random-effects model. RESULTS: The results of the pooled analysis of all 10 studies, 1 cohort and 9 case-control studies, indicated that maternal folate intake was inversely associated with CBSCT risk (OR 0.77; 95% CI 0.67-0.88, p < 0.001; I2 = 51.22%, p = 0.001). Separate analyses on the basis of the source of folate (folic acid supplementation, dietary folate) and in relation to the timing of exposure (before pregnancy, during pregnancy) found that folic acid supplementation was associated with an approximately 23% reduction in -CBSCT risk (OR 0.77, 95% CI 0.66-0.90, p = 0.001; I2 = 53.18%, p = 0.001) and consumption during pregnancy was associated with an approximately 20% reduction in CBSCT risk (OR 0.80, 95% CI 0.67-0.97, p = 0.020; I2 = 62.48%, p < 0·001). CONCLUSIONS: Maternal consumption of folic acid is associated with a reduced risk of CBSCT. Further investigations are necessary to increase the reliability of the results and estimate the relationship between dose-response and the best outcome.

Surgical outcomes of spinal cord and cauda equina ependymoma: Postoperative motor status and recurrence for each WHO grade in a multicenter study.

BACKGROUND: The goals of the study are to analyze postoperative outcomes and recurrence in cases of spinal cord and cauda equina ependymoma in each World Health Organization (WHO) Grade, and to examine the influence of extent of surgical removal on prognosis. Spinal ependymoma has a relatively high frequency among intramedullary spinal cord tumors. The tumor is classified in WHO guidelines as grades I, II, and III, but few studies have examined postoperative prognosis based on these grades. METHODS: The records of 80 patients undergoing surgery for spinal cord and cauda equina ependymoma were examined in a multicenter study using a retrospective database. Neurological motor status, pathological type, extent of resection, and tumor recurrence were evaluated. RESULTS: The histopathological types were grade I in 23 cases (myxopapillary: 21, subependymoma: 2), grade II in 52 cases, and grade III in 5 cases (including all anaplastic cases). Total resection was performed in 60 cases (83%), and eight cases had recurrence, including 2 in WHO grade I, 2 in grade II, and 4 in grade III. The 5-year recurrence-free survival rates were 90%, 91%, and 20% in grades I, II and III, respectively. Adjuvant radiotherapy for the local site was performed in 8 cases, including 3 in grade I and 5 in grade III; however, 4 of the 5 grade III cases (80%) had recurrence after radiotherapy. Among 59 patients with normal ambulation or independence without external assistance (McCormick Grade I or II), 53 (90%) maintained the same mobility after surgery. In cases that underwent total resection, the recurrence rate was significantly lower (p < 0.01). A good preoperative motor status also resulted in significantly better postoperative recovery of motor status (p < 0.05). CONCLUSIONS: Total resection of spinal cord and cauda equina ependymoma leads to postoperative motor recovery and may reduce tumor recurrence. Therefore, early surgery for this tumor is recommended before aggravation of paralysis.

Clinical presentation and outcomes for adult ependymoma patients.

BACKGROUND: Outcomes projects can be a catalyst for determining disease- and treatment-related consequences for patients with rare tumors. The Adult Ependymoma Outcomes (AEO) survey uses self-reported experience to evaluate how this tumor affects patient groups throughout the illness trajectory. METHODS: Patients completed the AEO survey via a Web-based portal. The survey included questions on treatment, tumor recurrence, and current health status; the MD Anderson Symptom Inventory Brain Tumor and Spine Tumor modules; and the Medical Outcomes Study 36-Item Short Form Health Survey (SF-36). RESULTS: The sample included 264 participants (57% female) with a median age of 46 years (range, 18-77 years). Radiation treatment was commonly used for patients who had brain involvement (χ2 (1) = 20.7; P < .001), underwent a partial resection (43%; χ2 (3) = 15.4; P < .001), or had a grade 3 tumor (41%; χ2 (2) = 18.8; P < .001). Recurrence occurred in a small group (29%), with grade 1 tumor patients 2.6 times more likely and grade 3 tumor patients 2.5 times more likely to experience recurrence than those with grade 2 tumors. Spine tumor patients had a higher symptom burden (mean, 2.8; scale, 0-10) than brain tumor patients (t(247) = -4.0), and they reported more moderate to severe symptoms (rating ≥ 5; 29%) than their counterparts (18%). Within the physical health portion of the SF-36, spine tumor patients reported worse health with respect to bodily pain (t(249) = 6.8; P < .001), physical functioning (t(252) = 4.1; P < .001), and vitality (t(202.2) = 3.0; P < .003). CONCLUSIONS: These results demonstrate the feasibility of implementing outcomes projects that report on the clinical and demographic characteristics of a rare patient population, and they underscore the importance of outcomes data in understanding disease-related issues. Cancer 2017;123:494-501. © 2016 American Cancer Society.

Natural history of intramedullary spinal cord ependymoma in patients preferring nonoperative treatment.

Surgical resection of intramedullary spinal cord ependymoma still remains the standard of care but is challenging and occasionally associated with poor outcome. The aim of this study is therefore to provide additional information regarding the natural history of conservatively treated symptomatic intramedullary spinal cord ependymoma. Retrospective, single center review of all patients with intramedullary spinal cord ependymoma treated conservatively (wait and see) between 1980 and 2016. The neurological outcomes at first presentation, as well as in long-term follow-up, were assessed using the modified McCormick Disability Scale and modified Rankin Scale. Thirteen of 41 patients were managed conservatively and were included in the study. Mean age at the admission was 49 years. There were seven women and six men. All patients were symptomatic at the time of presentation. The mean follow-up from admission to the last neurological examination was 47.9 months. The mean modified McCormick score in conservatively treated patients was 1.3 at admission and 1.6 (p = 0.3) at last follow-up. There was no significant neurological detoriation over time in conservatively managed patients as assessed by the modified Rankin Scale at first presentation and last follow-up (mRS scores of 0-2, 100 vs 92%; p = 0.9). This cohort of conservatively managed patients with symptomatic intramedullary spinal cord ependymoma was clinically stable throughout the follow-up period. Our data provide additional information for counseling patients with intramedullary spinal cord tumors who chose a nonoperative treatment.