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At least 127 records · Page 7Linked to original sources
PubMed2003

Optic pathway gliomas in neurofibromatosis type 1: the effect of presenting symptoms on outcome.

Children with neurofibromatosis type 1 (NF1) may present with optic pathway gliomas (OPG) that can progress to visual loss or other neurologic symptoms. These tumors may become evident either as a result of patient signs or symptoms or as an incidental finding on "baseline" neuroimaging studies. In an attempt to determine if there were differences between symptomatic and asymptomatic children with OPG, a retrospective cohort study of ninety children with NF1 and OPG was performed using data from two large NF1 referral centers. Age at diagnosis, presenting symptoms, tumor location, associated features, and clinical response were assessed for children who were initially symptomatic from their OPG (n = 51) and compared to similar data of asymptomatic children whose tumors were incidentally discovered by MRI (n = 39). There were no differences in age at presentation, tumor location, NF1-associated symptoms, or clinical response between the groups. Initially symptomatic children were much more likely to require treatment (OR: 14.8, 95% CI [1.9-116.7]) than those with incidentally discovered, asymptomatic OPG. Although 36% of OPG were diagnosed in children over the age of 6 years, none received prior neuroimaging and only two children had previously normal eye examinations, suggesting that the vast majority of OPG in this group were longstanding, undiagnosed tumors. Based on these findings, we do not advocate "baseline" MRI in children with NF1, but strongly recommend that all children of the age 10 years and younger with NF1 have complete annual ophthalmologic evaluations.

Child↗
PubMed1994

Optic pathway glioma infiltrating into somatostatinergic pathways in a young boy with gigantism. Case report.

The authors report gigantism in a 16-month-old boy with an extensive optic pathway glioma infiltrating into somatostatinergic pathways, as revealed by magnetic resonance imaging and immunocytochemical studies. Stereotactic biopsies of areas showing hyperintense signal abnormalities on T2-weighted images in and adjacent to the involved visual pathways provided rarely obtained histological correlation of such areas. The patient received chemotherapy, which resulted in reduction of size and signal intensity of the tumor and stabilization of vision and growth velocity.

Astrocytoma↗
PubMed2006

Optic pathway glioma: outcome and prognostic factors in a surgical series.

OBJECTS: The goals of this study were to evaluate the surgical outcomes of optic pathway glioma (OPG) and to analyze the prognostic factors related to the progression-free survival. MATERIALS AND METHODS: A retrospective review was conducted on 33 patients who underwent surgery for OPG; these included 15 male and 18 female patients with a mean age of 8.3 years. The mean duration of follow-up was 52 months. RESULTS AND CONCLUSIONS: The preservation rate of ipsilateral vision was 25%, while that of contralateral vision was 83% (P<0.001). There was no remarkable endocrine improvement after surgery. The overall and progression-free survival rates at 5 years were 93.6 and 52.4%, respectively. In our study, the predictors for tumor progression were children younger than 5 years of age (p=0.023) and of female gender (p=0.022). Because of the variable course of OPG, treatment policy should be optimized individually according to patient's status.

Adolescent↗
PubMed1976

The optic pathway in neurosarcoidosis.

A case is reported of a patient, age 40 yr, with progressive hypothalamic and visual failure. Autopsy revealed widespread multisystemic sarcoidosis with involvement of the central nervous system. The intracranial portion of the optic pathway was diffusely infiltrated by sarcoid granulomas. Comparison of this case with previously reported autopsy and biopsy findings leads to the following conclusions: 1) The intraocular, intraorbital, and intracranial parts of the optic nerves may be involved independently; 2) lesions of the nerve head and of the retrobulbar part of the nerve may be unilateral; and 3) involvement of the intracranial portion is usually bilateral and forms part of more widespread sarcoidosis of the central nervous system.

Adult↗
PubMed2006

Prognostic factors for progression of childhood optic pathway glioma: a systematic review.

A systematic literature review was carried out to evaluate best existing evidence on prognostic factors for progression of childhood optic pathway glioma. Databases were searched for relevant articles and articles selected independently by two authors. Information about study design, population, treatment, outcome and prognostic analysis were abstracted and the quality of each article was assessed. A total of 23 articles met the inclusion criteria. Many studies had important methodological limitations, regarding external and internal validity. Eleven studies evaluated possible prognostic factors in a multivariate analysis. Three high-quality studies indicated age<1 year as an independent prognostic factor for a worse progression-free survival. Three studies with multivariate analysis, including one high-quality study, found that children with neurofibromatosis type 1 (NF-1) have a better progression-free survival than those without NF-1. Two studies with multivariate analysis found tumour site to be a prognostic factor, both with some methodological limitations. In conclusion, this systematic review demonstrates that only a few of the prognostic factors proposed have been proven to be clinically relevant. Age<1 year is a clear and independent prognostic factor for progression-free survival. Other prognostic factors, such as NF-1, tumour site and others, are suggested, but are still without solid evidence and need further high-quality studies to be clearly proven.

Disease Progression↗
PubMed2005

Fractionated stereotactic radiotherapy of optic pathway gliomas: tolerance and long-term outcome.

PURPOSE: To evaluate the effectiveness and toxicity of fractionated stereotactically guided radiotherapy (FSRT) in the management of optic glioma. METHODS AND MATERIALS: Fifteen patients with optic pathway gliomas were treated with FSRT at our institution between 1990 and 2003. A median target dose of 52.2 Gy (range, 45.2-57.6 Gy) was applied using a median fractionation of 5 fractions of 1.8 Gy weekly using a linear accelerator. RESULTS: The median follow-up time was 97 months (range, 8-151 months). Of the 15 patients, 1 died of tumor progression during the follow-up period. The progression-free survival rate at 3 and 5 years was 92% and 72%, respectively. The median overall survival after FSRT was 90 months (range, 8-151 months). The 5-year survival rate after FSRT was 90%. We did not observe secondary malignancies. CONCLUSION: Fractionated stereotactic radiotherapy was safe and well tolerated in all patients. The good tumor control and the potential of sparing normal brain tissue, especially the pituitary gland in lesions involving the optic chiasm, permit effective treatment of patients with optic nerve gliomas. Longer follow-up is needed to assess the incidence of late effects fully.

Adolescent↗
PubMed1987

Electrophysiological detection of "silent" plaques in the optic pathways.

The incidence of a delayed P100 component of the VEP after checkerboard stimulation in probable or possible multiple sclerosis (MS) without history, signs or symptoms of optic neuritis is not significantly different from that found in other neurological disorders in which the visual system is unaffected. This reduces the diagnostic validity of a delayed P100 as evidence of "silent" plaques in the optic pathway, at least in suspected MS. The use of grating increases the VEP sensitivity in the MS group, but it still leaves more than a 30% chance of error in attributing a delayed P100 to a demyelinating disorder. In this respect the discordant behaviour of checkerboard and grating responses may represent a useful clue.

Adult↗
PubMedDate not supplied

MR imaging of optic pathways in patients with neurofibromatosis.

Twenty-one patients with documented neurofibromatosis had MR examinations to evaluate possible intracranial disease. In five cases the indication was a known or suspected optic glioma. Two patients were examined because of a history of seizures; the rest were examined as part of a baseline evaluation. Eighteen patients showed evidence of signal hyperintensity on T2-weighted images. Lesions involved the optic nerves, optic chiasm, optic tracts, lateral geniculate body, optic radiations, basal ganglia, periventricular white matter, cerebellar white matter, and dentate nucleus of the cerebellum. Comparison between MR and concurrent CT scans showed MR to be superior in demonstrating the posterior extent of optic-pathway gliomas. In addition, MR showed focal areas of hyperintensity in the basal ganglia, internal capsule, cerebellum, and/or white matter that were not detected on CT. Although we found MR to be superior to CT in detecting intracranial tumors in patients with neurofibromatosis, and in evaluating the extensive involvement of known lesions, the full clinical implications of our findings remain to be determined.

Adolescent↗
PubMed1989

Optic pathway gliomas in neurofibromatosis.

Two patients, a 5-year-old girl and a 3-year-old boy, with documented neurofibromatosis (NF-1) and visual pathway gliomas are reported. In the first patient, the tumor was a typical pilocytic astrocytoma confined to the proximal half of the right optic nerve just near the globe. The tumor was excised; after a follow-up of 7 years recurrence has not occurred. The second patient had a glioma of the left optic nerve, with involvement of the chiasm, optic tracts, probably lateral geniculate body, optic radiations and basal ganglia. Biventricular hydrocephalus, possibly due to the occlusion of Monro's foramina, was also noted. In this case, magnetic resonance imaging (MRI) was superior to computed tomography for visualizing the posterior extension of the lesions. The patient received X-ray therapy and after a follow-up of 3 years the clinical symptomatology remains stationary. It is suggested that the optic pathway involvement detected by MRI may represent a dysplastic element NF-1 rather than a truly neoplastic change.

Child, Preschool↗
PubMed2002

DNA topoisomerase IIalpha expression in optic pathway gliomas of childhood.

DNA topoisomerase IIalpha (Topo IIalpha) is linked to tumour cell growth and chemoresistance. We examined immunohistochemically Topo IIalpha expression levels in a series of 36 consecutive paediatric optic pathway glioma (OPG) patients. Topo IIalpha labelling index (LI) ranged from 0.0 to 11.6 and was significantly associated with patient age, with higher levels of Topo IIalpha in children < or = 3 years (P=0.031). Topo IIalpha expression did not correlate with patient survival. Topo IIalpha LI was not significantly increased in specimens of repeat surgery. Topo IIalpha LI closely correlated with MIB-1 LI (R=0.781, P<0.001). We conclude that Topo IIalpha expression correlates with tumour cell proliferation in paediatric OPGs. Assessment of cell proliferation, however, does not assist in refining prognostic predictions. Enhanced Topo IIalpha expression in children < or = 3.0 years suggests that Topo IIalpha-interfering anticancer compounds for adjuvant treatment of OPGs may be of particular benefit to young children.

Adolescent↗
PubMed2000

Carboplatin is effective therapy for young children with progressive optic pathway tumors: a Pediatric Oncology Group phase II study.

The Pediatric Oncology Group conducted a phase II study to evaluate the activity of carboplatin in children 5 years or younger with progressive optic pathway tumors (OPTs). Of the 51 patients accrued to this study, 1 was not eligible because the child was older than 6 years. Fifty patients were eligible and had either neuro-imaging or symptomatic evidence of progressive OPTs. Twenty-one of 50 had evidence of neurofibromatosis type I (NF-1). Therapy consisted of carboplatin 560 mg/m2 at 4-week intervals. Patients with stable disease or better after two courses were continued on therapy for 18 months or until progressive disease. Of the 50 eligible children, 39 had stable disease or better, and 34 completed the 18-month therapy. Our data are sufficient to conclude that the proportion of objective responses (complete, partial, or minor response or stable disease) exceeded 30% (P < 0.00001), and the approximate 95% confidence interval estimate of the objective response rate was 0.665 to 0.895. Twenty-one patients went off protocol because of progressive disease. Fifteen patients progressed during the 18-month therapy, and 6 patients progressed after completing therapy. Six children died with progressive disease. Major toxicities were neutropenia and thrombocytopenia, and 3 children experienced allergic reactions. Carboplatin is active and safe for the treatment of young children with progressive OPTs. The addition of other potentially active drugs may further increase the event-free survival for these children.

Antineoplastic Agents↗
PubMed1997

Endocrinologic disorders and optic pathway gliomas in children with neurofibromatosis type 1.

OBJECTIVE: To establish the prevalence of endocrinologic disorders in children with neurofibromatosis type 1 (NF1) and the relationship between these disorders and cerebral abnormalities on magnetic resonance imaging. DESIGN: A prospective follow-up study. Setting. A multidisciplinary neurofibromatosis clinic. PATIENTS: A total of 122 children diagnosed with NF1 according to diagnostic criteria set by the National Institutes of Health. RESULTS: Central precocious puberty (CPP) was diagnosed in 3 children and growth hormone deficiency (GHD) in 3 children. Optic pathway gliomas were observed in 15 children; in 9 of the 15 cases, the optic chiasm was involved. Of the 3 children with CPP, only 1 showed a chiasma glioma on magnetic resonance imaging. In 1 case with GHD, an optic chiasm glioma was detected on neuroimaging. Two of the 9 children with an optic chiasm glioma presented with CPP or GHD. CONCLUSIONS: It has been suggested that CPP in children with NF1 is found exclusively in the presence of a chiasma glioma. We conclude that chiasma glioma may not be obligatory in children with NF1 and CPP or GHD. Moreover, we report a prevalence of GHD in children with NF1 of 2.5%, which has not been established earlier.

Brain↗
PubMed2003

Progression-free survival in children with optic pathway tumors: dependence on age and the quality of the response to chemotherapy--results of the first French prospective study for the French Society of Pediatric Oncology.

PURPOSE: To evaluate a strategy aimed at avoiding radiotherapy during first-line treatment of children with progressive optic pathway tumors (OPT), by exclusively administering multiagent chemotherapy during 16 months. PATIENTS AND METHODS: Between 1990 and 1998, 85 children with progressive OPT were enrolled onto this multicenter nationwide trial. Chemotherapy alternating procarbazine plus carboplatin, etoposide plus cisplatin, and vincristine plus cyclophosphamide was given every 3 weeks. At the time of relapse or progression, second-line chemotherapy was authorized before recourse to radiotherapy. RESULTS: Objective response rate (partial response [PR] + complete response [CR]) to chemotherapy was 42%. Five-year progression-free survival (PFS) and overall survival rates were 34% and 89%, respectively. The 5-year radiotherapy-free survival rate was 61%. In the multivariate analysis of the 85 patients that entered onto the study, factors associated with the risk of disease progression were age younger than 1 year at diagnosis (P =.047) and absence of neurofibromatosis type 1 (P =.035). In the multivariate analysis of the 74 patients that remained on study after the first cycle of chemotherapy, factors associated with the risk of disease progression were age younger than 1 year at diagnosis (P =.0053) and no objective response to chemotherapy (P =.0029). Three-year PFS was 44% in infants < or = 1 year versus 66% in children older than 1 year. Three-year PFS was 53% in the absence of an objective response to chemotherapy versus 68% after a PR or CR. CONCLUSION: A significant proportion of children with OPT can avoid radiotherapy after prolonged chemotherapy. Deferring irradiation with chemotherapy protocols did not compromise overall survival of the entire population or visual function.

Age Factors↗
PubMed2001

Lethal X-linked microcephaly with dysmorphic features, bilateral optic pathway aplasia and normal eyes.

We describe a family, consisting of two brothers and a maternal uncle who died of an apparently identical condition, within a few days of birth, suggestive of an X-linked mode of inheritance. The propositus (the older sibling) was investigated in detail and showed the following clinical features: microcephaly, facial dysmorphism, malformations of hands and feet, and cryptorchidism. Examination of the brain revealed arhinencephaly, a primitive gyral pattern, arrested cortical maturation, absence of corticofugal tracts and corpus callosum, agenesis of the optic pathway with preserved eyes and oculomotor system, absent auditory pathway, agenesis of the pars compacta of the substantia nigra and severe hypoplasia of the cerebellum and its connections. This family belongs to the group of X-linked microcephalies and has some features in common with the Juberg-Marsidi syndrome. The fact that the CNS abnormalities were incompatible with life and the facial dysmorphic features were quite different makes it unlikely that the affected individuals in this family had Juberg-Marsidi syndrome. However, this does not exclude the possibility that more restricted anterior induction defects may occur in some X-linked microcephalies such as Juberg-Marsidi syndrome resulting in prolonged survival.

Eye↗
PubMed2006

Comparison of pattern visual-evoked potentials to perimetry in the detection of visual loss in children with optic pathway gliomas.

PURPOSE: We sought to compare visual evoked potentials (VEPs) with standard visual field testing in children with visual pathway gliomas. METHODS: Fifteen of 40 children with visual pathway gliomas verified on magnetic resonance imaging scan who cooperated with Goldmann visual field (GVF) and 3-channel VEPs were studied. GVFs were obtained in 25 eyes with adequate vision. VEP amplitudes, latencies, and signal-to-noise ratios (SNRs) were compared with control subjects. Four of the patients (5 eyes) also had Humphrey visual field testing. RESULTS: Twenty-two of 25 eyes had a field defect, 15 eyes showed a relative or absolute hemianopia, 7 eyes showed a central or generalized depression, and 3 eyes were normal. In hemianopic eyes, 87% showed a depression (GVF) or reduced sensitivity (Humphrey field) in the opposite hemifield. VEP amplitudes and SNRs, normally largest at the midline electrode, were significantly reduced in all eyes with visual field loss. By comparison, lateral electrodes showed significantly lower amplitudes and SNRs in patients and controls. Interhemispheric VEP asymmetry (>2:1 ratio) was seen in 67% of patients with hemianopia and 53% of controls. CONCLUSIONS: Reduction of amplitude and SNR at the midline VEP electrode was a sensitive indicator of visual field loss. Interhemispheric VEP asymmetry was not reliable in detection of a hemianopic field defect. VEPs can be a reliable and objective alternative for the detection of visual loss due to optic pathway glioma in children who are intolerant to visual field testing. We recommend the test protocol include pattern-onset and check reversal stimuli of at least one high and one low spatial frequency.

Adolescent↗