Diffuse intrinsic pontine glioma
- Mark W Kieran, MD, PhD
Mark W Kieran, MD, PhD
- Boston Children's Hospital
- Dana-Farber Cancer Institute
- Karen J Marcus, MD
Karen J Marcus, MD
- Associate Professor of Radiation Oncology
- Harvard Medical School
- Boston Children's Hospital
- Dana-Farber Cancer Institute
- Section Editors
- Jay S Loeffler, MD
Jay S Loeffler, MD
- Section Editor — Neurooncology
- Professor of Radiation Oncology
- Harvard Medical School
- Patrick Y Wen, MD
Patrick Y Wen, MD
- Section Editor — Neurooncology
- Professor of Neurology
- Harvard Medical School
- Amar Gajjar, MD
Amar Gajjar, MD
- Section Editor — Pediatric Neurooncology
- Scott and Tracie Hamilton Endowed Chair
- Director, Division of Neuro Oncology
- Co Leader Neurobiology and Brain Tumor Program
- Co Chair and Member, Department of Oncology
- St Jude Children’s Research Hospital
Brainstem gliomas are characterized by heterogeneous biologic behavior, ranging from low-grade tumors needing little treatment to those that are rapidly fatal despite aggressive therapy [1,2]. Prognosis and treatment depend upon both the clinical symptoms and their duration, the location of the tumor within the brainstem, and increasingly on the mutational profile.
Approximately 80 percent of pediatric brainstem gliomas arise within the pons, while the remaining 20 percent arise in the medulla, midbrain, or cervicomedullary junction (figure 1) [3-8]. The majority of pontine tumors are diffuse intrinsic brainstem gliomas, which are usually high-grade, locally infiltrative, and have a uniformly poor prognosis . Histologically, these tumors are usually anaplastic astrocytomas (World Health Organization [WHO] grade III) or glioblastoma (WHO grade IV). However, patients with WHO grade II tumors identified by biopsy do not have an improved prognosis. (See "Classification and pathologic diagnosis of gliomas".)
In contrast, most nonpontine tumors involving the cervicomedullary junction and tectum, as well as focal, cystic, and dorsal exophytic lesions, are low-grade astrocytomas, mostly grade I pilocytic astrocytomas . These are discrete, well-circumscribed tumors, often without evidence of locally invasive growth or edema . Approximately 10 to 20 percent of nonpontine gliomas will be high grade and are treated similarly to diffuse intrinsic pontine gliomas.
Diffuse intrinsic pontine gliomas will be reviewed here. Gliomas arising from other sites within the brainstem are discussed separately. (See "Focal brainstem glioma".)
Gliomas arising in the brainstem (midbrain, pons, and medulla oblongata) account for 10 to 20 percent of all central nervous system tumors in children. Brainstem gliomas are more common in children than adults [3,4,11,12]. In the United States, for example, there are approximately 300 pediatric cases and 100 adult cases reported each year. In children, the median age at diagnosis is five to nine years of age, and the incidence is approximately equal between males and females.
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- Histopathology and grade
- Molecular pathogenesis
- H3 K27M-mutant diffuse midline glioma
- CLINICAL PRESENTATION
- Differential diagnosis
- Radiation therapy
- Alternative radiation therapy techniques
- Investigational therapies
- SUMMARY AND RECOMMENDATIONS