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Pediatric low-grade gliomas: implications of the biologic era.

ABSTRACT: For the past decade, it has been recognized that pediatric low-grade gliomas (LGGs) and glial-neuronal tumors carry distinct molecular alterations with resultant aberrant intracellular signaling in the Ras-mitogen-activated protein kinase pathway. The conclusions and recommendations of a consensus conference of how best to integrate the growing body of molecular genetic information into tumor classifications and, more importantly, for future treatment of pediatric LGGs are summarized here. There is uniform agreement that molecular characterization must be incorporated into classification and is increasingly critical for appropriate management. Molecular-targeted therapies should be integrated expeditiously, but also carefully into the management of these tumors and success measured not only by radiographic responses or stability, but also by functional outcomes. These trials need to be carried out with the caveat that the long-term impact of molecularly targeted therapy on the developing nervous system, especially with long duration treatment, is essentially unknown.

SUBMITTER: Packer RJ

PROVIDER: S-EPMC5464436 | biostudies-literature | 2017 Jun

REPOSITORIES: biostudies-literature

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Pediatric low-grade gliomas: implications of the biologic era.

Packer Roger J RJ Pfister Stephan S Bouffet Eric E Avery Robert R Bandopadhayay Pratiti P Bornhorst Miriam M Bowers Daniel C DC Ellison David D Fangusaro Jason J Foreman Nicholas N Fouladi Maryam M Gajjar Amar A Haas-Kogan Daphne D Hawkins Cynthia C Ho Cheng-Ying CY Hwang Eugene E Jabado Nada N Kilburn Lindsay B LB Lassaletta Alvaro A Ligon Keith L KL Massimino Maura M Meeteren Schouten-van SV Mueller Sabine S Nicolaides Theo T Perilongo Giorgio G Tabori Uri U Vezina Gilbert G Warren Katherine K Witt Olaf O Zhu Yuan Y Jones David T DT Kieran Mark M

Neuro-oncology 20170601 6

For the past decade, it has been recognized that pediatric low-grade gliomas (LGGs) and glial-neuronal tumors carry distinct molecular alterations with resultant aberrant intracellular signaling in the Ras-mitogen-activated protein kinase pathway. The conclusions and recommendations of a consensus conference of how best to integrate the growing body of molecular genetic information into tumor classifications and, more importantly, for future treatment of pediatric LGGs are summarized here. There ...[more]

PMID: 27683733

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Project description:Purpose of reviewPediatric low-grade gliomas and glioneuronal tumors (pLGG) account for approximately 30% of pediatric CNS neoplasms, encompassing a heterogeneous group of tumors of primarily glial or mixed neuronal-glial histology. This article reviews the treatment of pLGG with emphasis on an individualized approach incorporating multidisciplinary input from surgery, radiation oncology, neuroradiology, neuropathology, and pediatric oncology to carefully weigh the risks and benefits of specific interventions against tumor-related morbidity. Complete surgical resection can be curative for cerebellar and hemispheric lesions, while use of radiotherapy is restricted to older patients or those refractory to medical therapy. Chemotherapy remains the preferred first-line therapy for adjuvant treatment of the majority of recurrent or progressive pLGG.Recent findingsTechnologic advances offer the potential to limit volume of normal brain exposed to low doses of radiation when treating pLGG with either conformal photon or proton RT. Recent neurosurgical techniques such as laser interstitial thermal therapy offer a "dual" diagnostic and therapeutic treatment modality for pLGG in specific surgically inaccessible anatomical locations. The emergence of novel molecular diagnostic tools has enabled scientific discoveries elucidating driver alterations in mitogen-activated protein kinase (MAPK) pathway components and enhanced our understanding of the natural history (oncogenic senescence). Molecular characterization strongly supplements the clinical risk stratification (age, extent of resection, histological grade) to improve diagnostic precision and accuracy, prognostication, and can lead to the identification of patients who stand to benefit from precision medicine treatment approaches. The success of molecular targeted therapy (BRAF inhibitors and/or MEK inhibitors) in the recurrent setting has led to a gradual and yet significant paradigm shift in the treatment of pLGG. Ongoing randomized trials comparing targeted therapy to standard of care chemotherapy are anticipated to further inform the approach to upfront management of pLGG patients.

Dataset Information

Pediatric low-grade gliomas: implications of the biologic era.

Publications

Pediatric low-grade gliomas: implications of the biologic era.

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