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Child's Nervous System : ChNS :... Jul 2009The objective of this study is to report our initial experience treating pediatric patients with central nervous system tumors using a frameless, optically guided linear...
OBJECTIVE
The objective of this study is to report our initial experience treating pediatric patients with central nervous system tumors using a frameless, optically guided linear accelerator.
MATERIALS AND METHODS
Pediatric patients were selected for treatment after evaluation by a multidisciplinary neuro-oncology team including neurosurgery, neurology, pathology, oncology, and radiation oncology. Prior to treatment, all patients underwent treatment planning using magnetic resonance imaging (MRI) and treatment simulation on a standard computed tomography scanner (CT). For CT simulation, patients were fitted with a customized plastic face mask with a bite block attached to an optical array with four reflective markers. After ensuring adequate reproducibility, these markers were tracked during treatment by an infra-red camera. All treatments were delivered on a Varian Trilogy linear accelerator. The follow-up period ranges from 1-18 months, with a median follow-up of 6 months.
RESULTS
Nine patients, ages ranging from 12 to 19 years old (median age 15 years old), with a variety of tumors have been treated. Patients were treated for juvenile pilocytic astrocytoma (JPA; n = 2), pontine low-grade astrocytoma (n = 1), pituitary adenoma (n = 3), metastatic medulloblastoma (n = 1), acoustic neuroma (n = 1), and pineocytoma (n = 1). We followed patients for a median of 12 months (range 3-18 months) with no in-field failures and were able to obtain encouraging toxicity profiles.
CONCLUSION
Frameless stereotactic optically guided radiosurgery and radiotherapy provides a feasible and accurate tool to treat a number of benign and malignant tumors in children with minimal treatment-related morbidity.
Topics: Adolescent; Astrocytoma; Brain Neoplasms; Child; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Medulloblastoma; Neoplasm Metastasis; Pineal Gland; Pinealoma; Pituitary Neoplasms; Prolactinoma; Radiosurgery; Radiotherapy; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 19326128
DOI: 10.1007/s00381-009-0840-8 -
Hong Kong Medical Journal = Xianggang... Feb 2013To investigate the pattern of malignancies in Chinese patients with neurofibromatosis type 1.
OBJECTIVE
To investigate the pattern of malignancies in Chinese patients with neurofibromatosis type 1.
DESIGN
Historical cohort study.
SETTING
Queen Mary Hospital and Duchess of Kent Children's Hospital in Hong Kong.
PATIENTS
Patients with neurofibromatosis type 1 seen between January 1995 and August 2011.
RESULTS
We identified 123 Chinese patients with neurofibromatosis type 1, diagnosed at a median age of 4.9 years (range, 0.1-16.1 years); 75 (61%) were males. They were followed up for a median of 9.7 years (range, 0.2-27.6 years). Most (80%) of the patients participated in our surveillance programme. Twelve patients developed malignancies at the ages of 0.8 to 41.6 years. These malignancies included: peripheral nerve sheath tumours (n=3), juvenile myelomonocytic leukaemia (n=2), optic nerve glioma (n=1), thalamic pilocytic astrocytoma (n=1), rhabdomyosarcoma (n=1), osteosarcoma (n=1), neuroblastoma (n=1), anaplastic large cell lymphoma (n=1), and breast carcinoma and subsequently carcinoma of the ampulla of Vater (n=1). Among them, three had their tumours (optic glioma, thalamic astrocytoma, sacral malignant peripheral nerve sheath tumour) initially detected by surveillance imaging. Four patients survived without disease progression, three are alive with active disease, the remaining five died (when aged 3 to 56 years) with progressive or relapsed malignancies. The latter patients died from a neuroblastoma, a juvenile myelomonocytic leukaemia, a malignant peripheral nerve sheath tumour, a lymphoma, and a second primary tumour (carcinoma of ampulla of Vater, at the age of 56 years). In neurofibromatosis type 1 patients with malignancy, overall 30-year survival was significantly shorter than in those without malignancy (35% vs 93%, P<0.001).
CONCLUSION
Chinese patients with neurofibromatosis type 1 are susceptible to different malignancies which contribute to mortality. These findings are similar to reports from overseas. Outcomes were unfavourable, except in patients having low-grade gliomas. Surveillance imaging may help early detection of deep-seated malignancies but the benefits accruing from such monitoring warrants prospective evaluation.
Topics: Adolescent; Child; Child, Preschool; China; Cohort Studies; Female; Follow-Up Studies; Hong Kong; Humans; Infant; Male; Neoplasms; Neurofibromatosis 1; Retrospective Studies; Survival Rate
PubMed: 23378354
DOI: No ID Found -
AJNR. American Journal of Neuroradiology Feb 2011Focal anaplasia characterized by T2 hypointensity, signal-intensity enhancement on postcontrast T1-weighted MR imaging and restricted water diffusion has been reported... (Comparative Study)
Comparative Study
Quantitative diffusion-weighted and dynamic susceptibility-weighted contrast-enhanced perfusion MR imaging analysis of T2 hypointense lesion components in pediatric diffuse intrinsic pontine glioma.
BACKGROUND AND PURPOSE
Focal anaplasia characterized by T2 hypointensity, signal-intensity enhancement on postcontrast T1-weighted MR imaging and restricted water diffusion has been reported in a patient with juvenile pilocytic astrocytoma. We identified T2(HOF) with these MR imaging characteristics in children with DIPG and hypothesized that these represent areas of focal anaplasia; and may, therefore, have increased perfusion properties and should be characterized by increased perfusion. Thus, we used DSC to investigate our hypothesis.
MATERIALS AND METHODS
We retrospectively reviewed the baseline MR imaging scans of 86 patients (49 girls, 37 boys; median age, 6.1 years; range, 1.1-17.6 years) treated for DIPG at our hospital (2004-2009). T2(HOF) with the described MR imaging characteristics was identified in 10 patients. We used a region of interest-based approach to compare the ADC, FA, rCBV, rCBF, and rMTT of T2(HOF) with those of the typical T2(HRT).
RESULTS
The ADC of T2(HOF) with the specified MR imaging characteristics was significantly lower than that of T2(HRT) (range, 0.71-1.95 μm(2)/ms versus 1.36-2.13 μm(2)/ms; P < .01); and the FA (range, 0.12-0.34 versus 0.07-0.24; P = .03) and rCBV (range, 0.4-2.62 versus 0.23-1.57; P = .01) values of T2(HOF)s were significantly higher.
CONCLUSIONS
Our data suggest that T2(HOF) in DIPG may represent areas of focal anaplasia and underline the importance of regional, rather than global, tumor-field analysis. T2(HOF) may be the ideal target when stereotactic biopsy of tumors that present with an inhomogeneous T2 signal intensity is considered.
Topics: Adolescent; Biopsy; Brain Stem Neoplasms; Child; Child, Preschool; Contrast Media; Diffusion Magnetic Resonance Imaging; Female; Glioma; Humans; Infant; Male; Necrosis; Retrospective Studies
PubMed: 21087935
DOI: 10.3174/ajnr.A2277 -
Journal of Clinical Oncology : Official... Jan 2011A phase I trial of lenalidomide was performed in children with recurrent, refractory, or progressive primary CNS tumors to estimate the maximum-tolerated dose (MTD) and...
PURPOSE
A phase I trial of lenalidomide was performed in children with recurrent, refractory, or progressive primary CNS tumors to estimate the maximum-tolerated dose (MTD) and to describe the toxicity profile and pharmacokinetics.
PATIENTS AND METHODS
Lenalidomide was administered by mouth daily for 21 days, repeated every 28 days. The starting dose was 15 mg/m(2)/d orally, and the dose was escalated according to a modified continuous reassessment method. Correlative studies included pharmacokinetics obtained from consenting patients on course 1, day 1, and at steady-state (between days 7 and 21).
RESULTS
Fifty-one patients (median age, 10 years; range, 2 to 21 years) were enrolled. Forty-four patients were evaluable for dose finding, and 49 patients were evaluable for toxicity. The primary toxicity was myelosuppression, but the MTD was not defined because doses up to 116 mg/m(2)/d were well-tolerated during the dose-finding period. Two objective responses were observed (one in thalamic juvenile pilocytic astrocytoma and one in optic pathway glioma) at dose levels of 88 and 116 mg/m(2)/d. Twenty-three patients, representing all dose levels, received ≥ six cycles of therapy. Pharmacokinetic analysis demonstrated that the lenalidomide area under the concentration-time curve from 0 to 24 hours and maximum plasma concentration increased with dosage over the range studied.
CONCLUSION
Lenalidomide was tolerable in children with CNS tumors at doses of 116 mg/m(2)/d during the initial dose-finding period. The primary toxicity is myelosuppression. Antitumor activity, defined by both objective responses and long-term stable disease, was observed, primarily in patients with low-grade gliomas.
Topics: Administration, Oral; Adolescent; Antineoplastic Agents; Area Under Curve; Central Nervous System Neoplasms; Child; Child, Preschool; Dose-Response Relationship, Drug; Drug Administration Schedule; Female; Humans; Lenalidomide; Male; Maximum Tolerated Dose; Recurrence; Thalidomide; Young Adult
PubMed: 21149652
DOI: 10.1200/JCO.2010.31.3601 -
Cureus Aug 2020We present a very rare case of chronic encapsulated intracerebral hematoma involving the septum pellucidum and the foramen of Monro that by location radiological...
We present a very rare case of chronic encapsulated intracerebral hematoma involving the septum pellucidum and the foramen of Monro that by location radiological appearance, and clinical history was mimicking a recurrent astrocytoma or a shunt-related foreign body granuloma. A young adult underwent the resection of a juvenile pilocytic astrocytoma as a child, and with a mass encasing the tip of an old non-functioning ventricular catheter, the differential diagnosis of shunt-related foreign body granuloma versus recurrent low-grade glioma was raised. Although chronic encapsulated intracerebral hematomas have been reported in the literature, the anatomical location of the lesion in the presented case was unique, with radiological and history findings also posing a peculiar diagnostic challenge. Chronic encapsulated intracerebral hematomas are benign entities that may also be found to involve deep and midline supra-tentorial structures usually not prone to spontaneous intraparenchymal hemorrhages. When symptomatic, surgical resection of the hematoma can be both diagnostic and curative.
PubMed: 32953346
DOI: 10.7759/cureus.9839 -
Journal of Neurosurgery. Pediatrics Oct 2014Low-grade glial and glioneuronal brain tumors are frequently encountered in the pediatric population and can be effectively treated by resection. The authors aimed to...
OBJECT
Low-grade glial and glioneuronal brain tumors are frequently encountered in the pediatric population and can be effectively treated by resection. The authors aimed to use imaging to evaluate how often tumors recurred and to determine if recurrences were associated with any clinical symptoms, along with the financial costs of imaging, in patients with radiographically proven gross-total resection (GTR) at Boston Children's Hospital. These data were assessed to propose guidelines regarding postoperative surveillance.
METHODS
The authors performed a retrospective cohort analysis of the Pediatric Brain Tumor Program database from 1993 to 2003 to identify patients with glial or glioneuronal tumors initially evaluated at Boston Children's Hospital. Among the 888 patients evaluated for any type of brain tumor during this period, 67 patients had WHO Grade I glial or glioneuronal lesions with radiographically proven GTR and available follow-up data. The frequency and timing of postoperative imaging was compared with the institutional protocol. Recurrence-free survival was calculated using the Kaplan-Meier method. Financial costs of imaging were available from 2001 to 2009 and were averaged to extrapolate the postoperative surveillance costs.
RESULTS
Among the 67 patients with GTR, 13 recurrences were detected radiographically with a mean time to recurrence of 32.4 months (range 2.9-128.5 months). The mean duration of follow-up after surgery was 6.6 years. The recurrence-free survival at 2 and 5 years after GTR for all low-grade glial and glioneuronal tumors was 0.90 (95% CI 0.82-0.97) and 0.82 (95% CI 0.73-0.92), respectively. No clinical symptoms were associated with any of the recurrences, and no deaths occurred. Under the institutional protocol of surveillance imaging, the estimated cost per recurrence at 5 years was $104,094 per patient. The proposed protocol would reduce the number of MR scans in the first 5 years from 10 to 5, providing a potential cost savings of $52,047 per recurrence.
CONCLUSIONS
Given the slow-growing, clinically asymptomatic nature of low-grade glial and glioneuronal tumors coupled with the financial and psychological costs of repeated imaging, the authors propose a postoperative surveillance MRI schedule that is less intensive than current institutional practice.
Topics: Adolescent; Boston; Brain Neoplasms; Child; Child, Preschool; Disease-Free Survival; Female; Follow-Up Studies; Humans; Infant; Kaplan-Meier Estimate; Male; Neoplasm Grading; Neoplasm Recurrence, Local; Neuroimaging; Postoperative Period; Retrospective Studies; Time Factors; Young Adult
PubMed: 25062303
DOI: 10.3171/2014.6.PEDS1321 -
Journal of Korean Neurosurgical Society Nov 2010Pilomyxoid astrocytoma (PMA) is a newly recognized variant of a pilocytic astrocytoma. This report describes a case of a pilomyxoid astrocytoma that occurred in the...
Pilomyxoid astrocytoma (PMA) is a newly recognized variant of a pilocytic astrocytoma. This report describes a case of a pilomyxoid astrocytoma that occurred in the opticohypothalamus. The patient was a 18-year-old girl who complained decreased visual acuity and visual field over a period of two years. Magnetic resonance imaging (MRI) showed an irregular lobulated tumor with heterogeneous enhancement at the suprasellar region involving the hypothalamus. The mass was partially removed via the subfrontal approach. Its pathology was confirmed to be PMA. Adjuvant chemotherapy with cisplatin and vincristine was started following tumor resection. After four cycles, the mass showed a partial response to the chemotherapy. Although long-term outcome is yet to be determined, the administration of combined cisplatin and vincristine treatment seems to be an effective regimen for a pilomyxoid astrocytoma.
PubMed: 21286484
DOI: 10.3340/jkns.2010.48.5.445 -
Brain Pathology (Zurich, Switzerland) Jul 2009In the present study, DNA from 28 pediatric low-grade astrocytomas was analyzed using Illumina HumanHap550K single-nucleotide polymorphism oligonucleotide arrays. A...
In the present study, DNA from 28 pediatric low-grade astrocytomas was analyzed using Illumina HumanHap550K single-nucleotide polymorphism oligonucleotide arrays. A novel duplication in chromosome band 7q34 was identified in 17 of 22 juvenile pilocytic astrocytomas and three of six fibrillary astrocytomas. The 7q34 duplication spans 2.6 Mb of genomic sequence and contains approximately 20 genes, including two candidate tumor genes, HIPK2 and BRAF. There were no abnormalities in HIPK2, and analysis of two mutation hot-spots in BRAF revealed a V600E mutation in only one tumor without the duplication. Fluorescence in situ hybridization confirmed the 7q34 copy number change and was suggestive of a tandem duplication. Reverse transcription polymerase chain reaction-based sequencing revealed a fusion product between KIAA1549 and BRAF. The predicted fusion product includes the BRAF kinase domain and lacks the auto-inhibitory N-terminus. Western blot analysis revealed phosphorylated mitogen-activated protein kinase (MAPK) protein in tumors with the duplication, consistent with BRAF-induced activation of the pathway. Further studies are required to determine the role of this fusion gene in downstream MAPK signaling and its role in development of pediatric low-grade astrocytomas.
Topics: Adolescent; Adult; Astrocytoma; Blotting, Western; Brain Neoplasms; Child; Child, Preschool; Chromosomes, Human, Pair 7; DNA Mutational Analysis; Female; Humans; In Situ Hybridization, Fluorescence; Infant; MAP Kinase Signaling System; Male; Oligonucleotide Array Sequence Analysis; Oncogene Proteins, Fusion; Polymorphism, Single Nucleotide; Proto-Oncogene Proteins B-raf; Reverse Transcriptase Polymerase Chain Reaction
PubMed: 19016743
DOI: 10.1111/j.1750-3639.2008.00225.x -
Journal of Nuclear Medicine : Official... May 1998This study evaluates the usefulness of PET for the preoperative evaluation of brain gliomas and methods of quantification of PET results. (Comparative Study)
Comparative Study
UNLABELLED
This study evaluates the usefulness of PET for the preoperative evaluation of brain gliomas and methods of quantification of PET results.
METHODS
Fifty-four patients with brain gliomas were studied by PET with 18F-fluorodeoxyglucose (FDG) (n = 45) and/or 11C-methionine (MET) (n = 41) before any treatment. Results of visual analysis, calculation of glucose consumption and five tumor-to-normal brain ratios for both tracers were correlated with two histologic grading systems and with follow-up.
RESULTS
Visual analysis (for FDG) and tumor-to-mean cortical uptake (T/MCU) ratio proved to be the best tools for the evaluation of PET results. Methionine was proven to be better than FDG at delineating low-grade gliomas. Tumor-to-mean cortical uptake ratios for FDG and MET were clearly correlated (r = 0.78), leading to the equation T/MCU(FDG) = 0.4 x T/MCU(MET). We showed a good correlation between FDG PET and histologic grading. MET uptake could not differentiate between low-grade and anaplastic astrocytomas but was significantly increased in glioblastomas. Low-grade oligodendrogliomas exhibited high uptake of FDG and MET, probably depending more on oligodendroglial cellular differentiation than on proliferative potential. Uptake was decreased in anaplastic oligodendrogliomas, probably due to dedifferentiation. Care must be taken with peculiar histologic subgroups, i.e., juvenile pilocytic astrocytomas and oligodendrogliomas, because of a discrepancy between high PET metabolism and low proliferative potential (good prognosis). Both tracers proved useful for the prediction of survival prognosis. Methionine proved slightly superior to FDG for predicting the histologic grade and prognosis of gliomas, despite the impossibility of differentiation between Grades II and III astrocytomas with MET. This superiority of MET could be explained by patient sampling (low number of Grade III gliomas submitted to examination with both tracers). The combination of both tracers improved the overall results compared to each tracer alone.
CONCLUSION
Both tracers are useful for the prediction of the histologic grade and prognosis. The apparent superiority of MET over FDG could be due to the small number of Grade III gliomas studied with both tracers.
Topics: Brain; Brain Neoplasms; Carbon Radioisotopes; Female; Fluorine Radioisotopes; Fluorodeoxyglucose F18; Glioma; Humans; Male; Methionine; Middle Aged; Radiopharmaceuticals; Survival Analysis; Tomography, Emission-Computed
PubMed: 9591574
DOI: No ID Found -
Journal of Korean Medical Science Jun 1989A total of 697 cases of intracranial and intraspinal tumors was obtained from the pathology file of Seoul National University Hospital and Children's Hospital during the... (Comparative Study)
Comparative Study
A total of 697 cases of intracranial and intraspinal tumors was obtained from the pathology file of Seoul National University Hospital and Children's Hospital during the period of 8 years from 1980 to 1987. These tumors were classified according to WHO classification. This study was performed to understand the recent trend of the relative frequency of the central nervous system tumors among Koreans and to compare it with the previous studies in Korea and other countries. There were 663 intracranial tumors and 34 intraspinal tumors. More common intracranial tumors were pituitary adenoma, meningioma, astrocytoma and medulloblastoma, each representing 23.4%, 20.8%, 11.8%, and 5.6%, respectively. In juvenile age group (under 15 years of age), medulloblastoma, astrocytoma, ependymoma and craniopharyngioma were more commonly encountered to be 25.6%, 21.6%, 13.6% and 12%, respectively. Both sexes were equally affected among adult group, but male preponderance was observed among juvenile group (1.49:1). Nine cases of primitive neuroectodermal tumor, a unique tumor which is not listed in WHO classification, were observed and all of them occurred before the age of 20. There were 27 metastatic tumors. Our previous study encompassing previous 17 years, 1963 to 1979, showed similar overall results except for intraspinal tumors that were more commonly encountered in previous series.
Topics: Adult; Age Factors; Brain Neoplasms; Female; Humans; Incidence; Korea; Male; Middle Aged; Neoplasm Metastasis; Sex Factors; Spinal Cord Neoplasms
PubMed: 2597364
DOI: 10.3346/jkms.1989.4.2.77