Journal of Child Neurology current issue

Child Neurologists Should Be Interested In Brain Tumors!

Brumback, R. A. — Mon, 19 Oct 2009 13:48:07 PDT

Introduction to a Special Issue on Pediatric Neuro-Oncology

Fangusaro, J., Chi, S. — Mon, 19 Oct 2009 13:48:07 PDT

Neuroimaging of Pediatric Brain Tumors: From Basic to Advanced Magnetic Resonance Imaging (MRI)

Panigrahy, A., Bluml, S. — Mon, 19 Oct 2009 13:48:07 PDT

In this review, the basic magnetic resonance concepts used in the imaging approach of a pediatric brain tumor are described with respect to different factors including understanding the significance of the patient’s age. Also discussed are other factors directly related to the magnetic resonance scan itself including evaluating the location of the tumor, determining if the lesion is extra-axial or intra-axial, and evaluating the contrast characteristics of the lesion. Of note, there are key imaging features of pediatric brain tumors, which can give information about the cellularity of the lesion, which can then be confirmed with advanced magnetic resonance imaging (MRI) techniques. The second part of this review will provide an overview of the major advanced MRI techniques used in pediatric imaging, particularly, magnetic resonance diffusion, magnetic resonance spectroscopy, and magnetic resonance perfusion. The last part of the review will provide more specific information about the use of advanced magnetic resonance techniques in the evaluation of pediatric brain tumors.

The Evolving Role of Surgery in the Management of Pediatric Brain Tumors

Souweidane, M. M. — Mon, 19 Oct 2009 13:48:07 PDT

Surgery is an integral component and typically the first line of therapy for children with central nervous system tumors. The outcome with regard to surgical morbidity and disease control can be greatly influenced by the initial care that these children receive. Conventional aims of neurosurgery including tumor removal, management of hydrocephalus, and diagnostic sampling have been radically modified with innovative technologies such as navigational guidance, functional mapping, endoscopic surgery, second-look surgery, and physiologic imaging. The overall role of the pediatric neurosurgeon in caring for children with nervous system tumors is also expanding to include unconventional responsibilities including disease staging, tissue procurement, and drug delivery. It is thus anticipated that the pediatric neurosurgeon will be increasingly relied upon for oncologic therapeutic strategies and should thus remain abreast of forthcoming information and technologies.

Histology and Molecular Pathology of Pediatric Brain Tumors

Pfister, S., Hartmann, C., Korshunov, A. — Mon, 19 Oct 2009 13:48:07 PDT

In recent years, brain tumors have become the single most frequent cause of cancer-related mortality in children, although their frequency is approximately 50% less than leukemia. According to the classification of the World Health Organization, histopathological diagnosis is of paramount importance for clinicians to choose the most appropriate treatment option and tailor treatment intensity to disease risk. However, histopathological assessment is often difficult, and adding molecular information to classic neuropathological analyses may help ensure diagnostic accuracy, improve risk stratification of patients within a given tumor entity, and help in identifying novel therapeutic targets for an individualized treatment approach. Therefore, this review focuses both on established histopathology as well as on molecular features in the most important brain tumors in children.

Radiation Therapy for Pediatric Central Nervous System Tumors

Hoffman, K. E., Yock, T. I. — Mon, 19 Oct 2009 13:48:07 PDT

Radiation therapy is an important component of treatment of many pediatric central nervous system tumors. The radiation treatment target is determined by tumor histology, extent of disease, anticipated pattern of spread, and expected pattern of failure. Children cured of their tumors live to experience the long-term sequelae of radiation treatment, including developmental, neurocognitive, neuroendocrine, and hearing late effects. The development of more conformal radiation techniques has decreased inadvertent radiation dose to normal tissues and should decrease long-term treatment sequelae that are the result of normal tissue radiation. Intensity-modulated radiation therapy improves treatment conformity and decreases high dose to nearby normal tissues; however, it delivers a larger volume of low- and intermediate-dose radiation. Proton radiation eliminates exit dose to normal tissues, thereby eliminating approximately 50% of unnecessary radiation to normal tissues. The long-term clinical benefits of proton radiation in the pediatric population are just beginning to be reported in the literature.

Pediatric Low-Grade Gliomas

Sievert, A. J., Fisher, M. J. — Mon, 19 Oct 2009 13:48:07 PDT

Pediatric low-grade gliomas encompass a heterogeneous set of tumors of different histologies. Cerebellar pilocytic astrocytomas occur most frequently followed by supratentorial diffuse fibrillary astrocytomas. Recent research has implicated activation of the RAS/RAF/MEK pathway in tumorigenesis of these tumors. Surgery is the mainstay of therapy. Overall survival rates for patients whose tumors are completely resected are 90% or greater, 10 years from diagnosis. Conversely, most optic pathway/hypothalamic, deep midline, and brain stem gliomas have minimal potential for resection; these tumors can be difficult to treat and deserve special attention. Combination chemotherapy is currently recommended as front-line adjuvant treatment for progressive or recurrent tumors. Second-line radiotherapy can also improve overall survival but is associated with more frequent and significant neurocognitive, endocrine, and other long-term toxicities.

Pediatric High-Grade Gliomas and Diffuse Intrinsic Pontine Gliomas

Fangusaro, J. — Mon, 19 Oct 2009 13:48:07 PDT

Pediatric high-grade gliomas represent approximately 10% of all pediatric brain tumors. Similar to adult high-grade gliomas, they behave very aggressively, and these children have a very poor prognosis despite a variety of therapies that include chemotherapy and radiotherapy. In this review, we present an overview of both pediatric high-grade gliomas and diffuse intrinsic pontine gliomas with a focus on their epidemiology, etiology, presentation, prognostic factors, biology, treatment modalities, outcomes, and future research directions.

Medulloblastoma

Dhall, G. — Mon, 19 Oct 2009 13:48:07 PDT

Medulloblastoma is the most common malignant brain tumor in children. Patients with medulloblastoma are stratified into ‘‘standard’’ and ‘‘high’’ risk categories based on age at diagnosis, degree of surgical resection, and disease spread. In children older than 3 years of age, the long-term survival can be achieved in approximately 85% of standard risk patients and 70% of high risk patients with a combination of chemotherapy and irradiation. Younger children, particularly infants, are at a significantly higher risk of side-effects of treatment. Despite tremendous progress in the field of molecular biology of medulloblastoma, much remains to be achieved in understanding the pathogenesis, critical pathways responsible for medulloblastoma, and molecular risk stratification, and in devising treatment strategies with even better survival and less long-term sequelae.

Ependymoma: An Update

Zacharoulis, S., Moreno, L. — Mon, 19 Oct 2009 13:48:07 PDT

The authors provide an update on most issues related to biology, diagnosis, and treatment of children with ependymoma based on a literature review. Ependymoma is the third most common brain tumor in children and overall survival ranges from 24% to 75% at 5 years. The extent of surgical resection remains the principal risk factor that clearly influences outcome. The influence of age, location, grade, or stage has proved to be more controversial. Current standard therapy includes surgical resection and radiotherapy. Chemotherapy has a role in infants to avoid/delay radiotherapy and can be helpful to improve resectability. About half of patients will experience relapse, and outcome is dismal. New radiation modalities, reirradiation, chemotherapy, or targeted agents have been tested with promising results. Results of multi-institutional clinical trials are awaited to determine the best first-line treatment, while results of early phase I/ II trials will explore directed therapies based on new biologic factors.

Central Nervous System Germ Cell Tumors: Classification, Clinical Features, and Treatment With a Historical Overview

Fujimaki, T. — Mon, 19 Oct 2009 13:48:07 PDT

Central nervous system germ cell tumors are neoplasms that affect children and young adults. They are subclassified into germinoma and nongerminomatous germ cell tumors. The latter include teratoma (mature teratoma, immature teratoma, teratoma with malignant transformation), choriocarcinoma, embryonal carcinoma, yolk sac tumors, and mixtures of these entities. Germinoma with syncytiotrophoblastic giant cells is a variant of germinoma. Germinomas respond well to radiation therapy, but late sequelae due to irradiation have been reported. The results of radiation treatment alone for nongerminomatous germ cell tumor are not satisfactory. Combination radiochemotherapy has been applied, and this yields a good outcome with less toxicity for germinomas and better survival of nongerminomatous germ cell tumors. This article also discusses other issues, including the controversy regarding spinal irradiation and the treatment of recurrent disease.

Neurologic Sequelae of Brain Tumors in Children

Ullrich, N. J. — Mon, 19 Oct 2009 13:48:07 PDT

Neurologic signs and symptoms are often the initial presenting features of a primary brain tumor and may also emerge during the course of therapy or as late effects of the tumor and its treatment. Variables that influence the development of such neurologic complications include the type, size, and location of the tumor, the patient’s age at diagnosis, and the treatment modalities used. Heightened surveillance and improved neuroimaging modalities have been instrumental in detecting and addressing such complications, which are often not appreciated until many years after completion of therapy. As current brain tumor therapies are continually refined and newer targeted therapies are developed, it will be important for future cooperative group studies to include systematic assessments to determine the incidence of neurologic complications and to provide a framework for the development of novel strategies for prevention and intervention.

Late Effects of Therapy for Pediatric Brain Tumor Survivors

Turner, C. D., Rey-Casserly, C., Liptak, C. C., Chordas, C. — Mon, 19 Oct 2009 13:48:07 PDT

Approximately 2 of every 3 of all pediatric patients with brain tumors will be long-term survivors. However, there is a steep cost for pediatric brain tumor survivors, and the group as a whole faces significantly more late effects than many other survivors of pediatric cancers. Most of these effects can be attributed to direct neurologic damage to the developing brain caused by the tumor and its removal, the long-term toxicity of chemotherapy, or the effects of irradiation on the central nervous system. The late effects experienced by childhood brain tumor survivors involve multiple domains. This article will review the significant late effects that occur within the medical, neurocognitive, psychosocial, and economic domains of the survivorship experience. We conclude by discussing how the late effects in different domains often coexist and can create a complex set of obstacles that pose significant challenges for a survivor of a pediatric brain tumor on a daily basis.

Diagnosis and Treatment of Childhood Brain Tumors: Current Perspectives

Pollack, I. F. — Mon, 19 Oct 2009 13:48:07 PDT

JCN Calendar of Events

Mon, 19 Oct 2009 13:48:07 PDT