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  • Journal of Neurosurgery Publishing Group (JNSPG)  (21)
  • 1
    In: Journal of Neurosurgery: Pediatrics, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 17, No. 3 ( 2016-03), p. 298-302
    Abstract: Primary CNS sarcomas are very rare pediatric tumors with no defined standard of care. METHODS This study was a retrospective review of children diagnosed with a primary CNS sarcoma and treated at 2 Canadian tertiary care centers between 1995 and 2012. This report focuses on patients with cerebral hemispheric tumor location due to their specific clinical presentation. RESULTS Fourteen patients with nonmetastatic primary CNS sarcoma were identified; in 9 patients, tumors were located in the cerebral hemisphere and 7 of these patients presented with intratumoral hemorrhage. One infant who died of progressive disease postoperatively before receiving any adjuvant therapy was not included in this study. The final cohort therefore included 8 patients (4 males). Median patient age at diagnosis was 11.8 years (range 5.8–17 years). All tumors were located in the right hemisphere. Duration of symptoms prior to diagnosis was very short with a median of 2 days (range 3–7 days), except for 1 patient. Three (37.5%) patients had an underlying diagnosis of neurofibromatosis Type 1 (NF1). Gross-total resection was achieved in 5 patients. The dose of focal radiation therapy (RT) ranged between 54 Gy and 60 Gy. Concomitant etoposide was administered during RT. ICE (ifosfamide, carboplatin, etoposide) chemotherapy was administered prior to and after RT for a total of 6–8 cycles. Seven of the 8 patients were alive at a median time of 4.9 years (range 1.9–17.9 years) after treatment. CONCLUSIONS In this retrospective series, patients with primary CNS sarcomas located in the cerebral hemisphere most commonly presented with symptomatic acute intratumoral hemorrhage. Patients with NF1 were overrepresented. The combination of adjuvant ICE chemotherapy and focal RT provided encouraging outcomes.
    Type of Medium: Online Resource
    ISSN: 1933-0707 , 1933-0715
    RVK:
    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 2016
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  • 2
    Online Resource
    Online Resource
    Journal of Neurosurgery Publishing Group (JNSPG) ; 2007
    In:  Journal of Neurosurgery: Pediatrics Vol. 107, No. 4 ( 2007-10), p. 318-323
    In: Journal of Neurosurgery: Pediatrics, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 107, No. 4 ( 2007-10), p. 318-323
    Type of Medium: Online Resource
    ISSN: 1933-0707
    RVK:
    Language: English
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 2007
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  • 3
    In: Journal of Neurosurgery, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 135, No. 4 ( 2021-10), p. 1139-1145
    Abstract: There is no consensus on the optimal clinical management of ventriculomegaly and hydrocephalus in patients with diffuse intrinsic pontine glioma (DIPG). To date, the impact on survival in patients with ventriculomegaly and CSF diversion for hydrocephalus in this population remains to be elucidated. Herein, the authors describe their institutional experience. METHODS Patients diagnosed with DIPG and treated with up-front radiation therapy (RT) at The Hospital for Sick Children between 2000 and 2019 were identified. Images at diagnosis and progression were used to determine the frontal/occipital horn ratio (FOR) as a method to measure ventricular size. Patients with ventriculomegaly (FOR ≥ 0.36) were stratified according to the presence of symptoms and categorized as follows: 1) asymptomatic ventriculomegaly and 2) symptomatic hydrocephalus. For patients with ventriculomegaly who did not require CSF diversion, post-RT imaging was also evaluated to assess changes in the FOR after RT. Proportional hazards analyses were used to identify clinical and treatment factors correlated with survival. The Kaplan-Meier method was used to perform survival estimates, and the log-rank method was used to identify survival differences between groups. RESULTS Eighty-two patients met the inclusion criteria. At diagnosis, 28% (n = 23) of patients presented with ventriculomegaly, including 8 patients who had symptomatic hydrocephalus and underwent CSF diversion. A ventriculoperitoneal shunt was placed in the majority of patients (6/8). Fifteen asymptomatic patients were managed without CSF diversion. Six patients had resolution of ventriculomegaly after RT. Of 66 patients with imaging at the time of progression, 36 (55%) had ventriculomegaly, and 9 of them required CSF diversion. The presence of ventriculomegaly at diagnosis did not correlate with survival on univariate analysis. However, patients with symptomatic hydrocephalus at the time of progression who underwent CSF diversion had a survival advantage (p = 0.0340) when compared to patients with ventriculomegaly managed with conservative approaches. CONCLUSIONS Although ventriculomegaly can be present in up to 55% of patients with DIPG, the majority of patients present with asymptomatic ventriculomegaly and do not require surgical interventions. In some cases ventriculomegaly improved after medical management with steroids and RT. CSF diversion for hydrocephalus at the time of diagnosis does not impact survival. In contrast, our results suggest a survival advantage in patients who undergo CSF diversion for hydrocephalus at the time of progression, albeit that advantage is likely to be confounded by biological and individual patient factors. Further research in this area is needed to understand the best timing and type of interventions in this population.
    Type of Medium: Online Resource
    ISSN: 0022-3085 , 1933-0693
    RVK:
    RVK:
    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 2021
    detail.hit.zdb_id: 2026156-1
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  • 4
    Online Resource
    Online Resource
    Journal of Neurosurgery Publishing Group (JNSPG) ; 2007
    In:  Journal of Neurosurgery: Pediatrics Vol. 106, No. 5 ( 2007-05), p. 363-367
    In: Journal of Neurosurgery: Pediatrics, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 106, No. 5 ( 2007-05), p. 363-367
    Type of Medium: Online Resource
    ISSN: 1933-0707
    RVK:
    Language: English
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 2007
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  • 5
    In: Journal of Neurosurgery: Pediatrics, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 21, No. 2 ( 2018-02), p. 145-152
    Abstract: Metastatic dissemination is a major treatment challenge and cause of death in patients with medulloblastoma. However, the influence of molecular biology on the pattern of metastatic dissemination at diagnosis is not known. In this study, the authors sought to define the location, pattern, and imaging characteristics of medulloblastoma metastases across subgroups at diagnosis. METHODS A consecutive cohort of patients with metastatic medulloblastoma at The Hospital for Sick Children and the University Hospital Motol, who underwent up-front MRI of the craniospinal axis, was assembled and allocated to subgroups using NanoString limited gene–expression profiling. Radiological characteristics (including location, morphology, size, diffusion restriction, and contrast enhancement) were discerned through a retrospective review. RESULTS Forty metastatic medulloblastomas were identified with up-front neuroimaging of the craniospinal axis: 5 sonic hedgehog (SHH), 16 Group 3, and 19 Group 4 metastases. Significant subgroup-specific differences were observed, particularly with respect to tumor location, size, and morphology. Group 3 metastases were most frequently laminar compared with a more nodular pattern in Group 4 (14 of 16 in Group 3 vs 8 of 19 in Group 4; p = 0.0004). Laminar metastases were not observed in patients with SHH medulloblastoma. Suprasellar metastases are highly specific to Group 4 (p = 0.016). Two of the 5 SHH cases had multifocal lesions in the cerebellum, raising the possibility that these were in fact synchronous primary tumors and not true metastases. A minority of patients with Group 4 metastases harbored metastatic deposits that did not enhance on MRI after contrast administration, often in patients whose primary tumor did not enhance. CONCLUSIONS The location, morphology, and imaging characteristics of metastatic medulloblastoma differ across molecular subgroups, with implications for diagnosis and management. This suggests that the biology of leptomeningeal dissemination differs among medulloblastoma subgroups.
    Type of Medium: Online Resource
    ISSN: 1933-0707 , 1933-0715
    RVK:
    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 2018
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  • 6
    In: Journal of Neurosurgery, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 137, No. 3 ( 2022-09-01), p. 807-812
    Abstract: The objective of this study was to describe the management of hydrocephalus in a cohort of pediatric patients with germinoma. METHODS The authors conducted a retrospective chart review of patients with germinoma and symptomatic hydrocephalus treated at the Hospital for Sick Children between 2002 and 2020. Descriptive data included tumor location, CSF diversion procedure (external ventricular drain [EVD], endoscopic third ventriculostomy [ETV] , ventriculoperitoneal [VP] shunt) and outcomes. The frontooccipital horn ratio (FOR) method was used to determine the presence of ventriculomegaly. RESULTS Of 39 patients with germinoma, 22 (73% male) had symptomatic hydrocephalus at diagnosis (11 pineal, 4 suprasellar, and 7 bifocal). Management of hydrocephalus included EVD (n = 5, 22.7%), ETV (n = 5, 22.7%), and combination ETV and EVD (n = 7, 31.8%), whereas 5 patients (22.7%) did not undergo surgical intervention. The median FOR at diagnosis was 0.42 (range 0.38–0.58), which correlated with moderate to severe ventriculomegaly. Carboplatin and etoposide–based chemotherapy induced fast tumor shrinkage, avoiding CSF diversion (n = 5) and resolving hydrocephalus with a transient EVD (n = 5). The median duration until EVD removal was 7 days (range 2–10 days). Two of 12 patients with EVD ultimately required a VP shunt. Kaplan-Meier overall survival was 100% and progression-free survival was 96.4% at a median follow-up of 10.4 years. CONCLUSIONS Timely initiation of chemotherapy is imperative to rapidly reduce tumor bulk in children with germinoma and limits the need for VP shunt insertions. In children in whom CSF diversion is required, hydrocephalus may be successfully managed with a temporary EVD ± ETV.
    Type of Medium: Online Resource
    ISSN: 0022-3085 , 1933-0693
    RVK:
    RVK:
    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 2022
    detail.hit.zdb_id: 2026156-1
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  • 7
    Online Resource
    Online Resource
    Journal of Neurosurgery Publishing Group (JNSPG) ; 2017
    In:  Journal of Neurosurgery: Pediatrics Vol. 20, No. 6 ( 2017-12), p. 549-555
    In: Journal of Neurosurgery: Pediatrics, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 20, No. 6 ( 2017-12), p. 549-555
    Abstract: Isolated optic nerve gliomas (IONGs) constitute a rare subgroup of optic pathway gliomas (OPGs). Due to the rarity of this condition and the difficulty in differentiating IONGs from other types of OPGs in most clinical series, little is known about these tumors. Currently, due to lack of evidence, they are managed the same as any other OPG. METHODS The authors conducted a multicenter retrospective cohort study aimed at determining the natural history of IONGs. Included were patients with clear-cut glioma of the optic nerve without posterior (chiasmatic/hypothalamic) involvement. At least 1 year of follow-up, 2 MRI studies, and 2 neuro-ophthalmological examinations were required for inclusion. RESULTS Thirty-six patients with 39 tumors were included in this study. Age at diagnosis ranged between 6 months and 16 years (average 6 years). The mean follow-up time was 5.6 years. Twenty-five patients had neurofibromatosis Type 1. During the follow-up period, 59% of the tumors progressed, 23% remained stable, and 18% (all with neurofibromatosis Type 1) displayed some degree of spontaneous regression. Fifty-one percent of the patients presented with visual decline, of whom 90% experienced further deterioration. Nine patients were treated with chemotherapy, 5 of whom improved visually. Ten patients underwent operation, and no local or distal recurrence was noted. CONCLUSIONS Isolated optic nerve gliomas are highly dynamic tumors. Radiological progression and visual deterioration occur in greater percentages than in the general population of patients with OPGs. Response to chemotherapy may be better in this group, and its use should be considered early in the course of the disease.
    Type of Medium: Online Resource
    ISSN: 1933-0707 , 1933-0715
    RVK:
    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 2017
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  • 8
    Online Resource
    Online Resource
    Journal of Neurosurgery Publishing Group (JNSPG) ; 2013
    In:  Journal of Neurosurgery: Pediatrics Vol. 12, No. 4 ( 2013-10), p. 325-327
    In: Journal of Neurosurgery: Pediatrics, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 12, No. 4 ( 2013-10), p. 325-327
    Abstract: Medulloblastoma is a highly malignant brain tumor of childhood. Although craniospinal dissemination within the subarachnoid space is common, invasion of the dural sinuses is rare. Here, the authors report on a 15-year-old girl who presented with a right cerebellar mass, obstructive hydrocephalus, and radiographic evidence of tumor invasion into the right transverse–sigmoid sinus junction. The patient underwent posterior fossa craniotomy, gross-total resection of the intraparenchymal component of the right cerebellar tumor, and coagulation of the tumor invading the transverse sinus. After pathological confirmation of anaplastic medulloblastoma, the patient underwent craniospinal radiation therapy and high-dose chemotherapy. At 2 years posttreatment, the child was neurologically intact with no radiographic evidence of residual disease or recurrence. The implications for disease prognosis and management are discussed.
    Type of Medium: Online Resource
    ISSN: 1933-0707 , 1933-0715
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    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 2013
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  • 9
    Online Resource
    Online Resource
    Journal of Neurosurgery Publishing Group (JNSPG) ; 2013
    In:  Journal of Neurosurgery: Pediatrics Vol. 12, No. 3 ( 2013-09), p. 235-240
    In: Journal of Neurosurgery: Pediatrics, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 12, No. 3 ( 2013-09), p. 235-240
    Abstract: In the face of increasing survival, quality of life (QOL) has become an important indicator of treatment success in children with posterior fossa brain tumors (PFBTs). The authors' objective was to assess the long-term QOL in survivors of PFBT. Methods The authors conducted a cross-sectional study of children who, between birth and age 18 years at diagnosis, had previously been treated at their institution for a PFBT. At the time of assessment for this study, children were between 5 and 19 years old and had received standard treatment for PFBT ending at least 6 months before the assessment. The QOL was measured with the Pediatric Quality of Life Inventory (PedsQL) generic score scales and the Health Utilities Index Mark 3 (HUI3). Multivariate analyses were used to assess several variables (patient related, treatment related, and socioeconomic) for association with QOL. Results A total of 62 children participated in the study (median age at assessment 11.9 years, interquartile range [IQR] 7.8–14.8, and median age at tumor diagnosis of 4.9 years, IQR 2.5–6.9). Median time since active treatment for their PFBT was 5.2 years (IQR 2.4–10.1). Tumor types included cerebellar pilocytic astrocytoma (45.2%), medulloblastoma (30.6%), ependymoma (11.3%), and brainstem astrocytoma (11.3%). Adjuvant therapy included chemotherapy (40.3%) or radiotherapy (14.5% focal and 21.0% craniospinal radiotherapy). Permanent treatment for hydrocephalus was required in 38.7% of the patients. Tumors recurred in 11.3%, requiring repeat treatment in these patients. The median HUI3 utility score was 0.91 (IQR 0.71–1.00) and the median PedsQL total score was 78.3 (IQR 64.1–92.4). Only the following variables were significantly associated with decreased QOL in multivariable model testing (all p 〈 0.05): need for permanent hydrocephalus treatment, large ventricle size, decreased family functioning, and lower family income. Conclusions As a group, long-term survivors of pediatric PFBT appear to have QOL indicators that are similar to those of the general population, although a reasonable minority of patients experience poor outcomes. Although several confounding variables likely remain in this retrospective study, important associations with QOL include the presence of hydrocephalus and socioeconomic factors. The study sample size, however, was limited and the presence of other important factors cannot be excluded.
    Type of Medium: Online Resource
    ISSN: 1933-0707 , 1933-0715
    RVK:
    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 2013
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  • 10
    Online Resource
    Online Resource
    Journal of Neurosurgery Publishing Group (JNSPG) ; 2009
    In:  Journal of Neurosurgery: Pediatrics Vol. 4, No. 3 ( 2009-09), p. 254-261
    In: Journal of Neurosurgery: Pediatrics, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 4, No. 3 ( 2009-09), p. 254-261
    Abstract: Intramedullary spinal cord low-grade gliomas (LGGs) are rare CNS neoplasms in pediatric patients, and there is little information on therapy for and outcome of these tumors in this population. Furthermore, most patient series combine adult and pediatric patients or high- and low-grade tumors, resulting in controversial data regarding optimal treatment of these children. To clarify these issues, the authors performed a regional population-based study of spinal cord LGGs in pediatric patients. Methods All pediatric patients with LGGs treated during the MR imaging era (1985–2007) were identified in the comprehensive database of the Hospital for Sick Children in Toronto. Data on demographics, pathology, treatment details, and outcomes were collected. Results Spinal cord LGGs in pediatric patients constituted 29 (4.6%) of 635 LGGs. Epidemiological and clinical data in this cohort were different than in patients with other spinal tumors and strikingly similar to data from pediatric patients with intracranial LGGs. The authors observed an age peak at 2 years and a male predominance in patients with these tumors. Histological testing revealed a Grade I astrocytoma in 86% of tumors. Although 5-year progression-free survival for the entire group was 48 ± 9%, all patients were alive at a median follow-up of 8.2 years. Five-year progression-free survival was 88 ± 13% for patients undergoing gross-total resection and 34 ± 11% for those undergoing all other therapies, respectively (p = 0.02). Chemotherapy and radiation therapy showed similar efficacy, achieving sustained tumor control in most patients. However, this excellent survival rate was associated with an 83% rate of significant neurological and orthopedic sequelae. Conclusions This study provides basic data on the incidence, clinical course, and outcome of spinal cord LGGs in pediatric patients. The similarities between spinal and intracranial LGGs in pediatric patients showing excellent survival but high morbidity suggest that a less aggressive approach may be the preferable treatment option for these patients.
    Type of Medium: Online Resource
    ISSN: 1933-0707 , 1933-0715
    RVK:
    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 2009
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