Rare Pediatrics News

Disease Profile

Childhood brain stem glioma

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Categories

Rare Cancers

Summary

Childhood brain stem glioma is a rare condition in which abnormal cells develop in the tissues of the brain stem (the part of the brain connected to the spinal cord). The condition can be benign (noncancerous) or malignant (cancerous). The severity of the condition and the associated signs and symptoms vary based on the size and location of the tumor and how quickly the tumor is growing. Common features include difficulty walking; loss of the ability to move one side of the body or face; vision or hearing problems; headaches (particularly in the morning); nausea and vomiting; unusual sleepiness; and behavioral changes. In most cases, the underlying cause of childhood brain stem glioma is unknown. Certain genetic conditions, such as neurofibromatosis type I, are associated with an increased risk of childhood brainstem glioma. Treatment varies but may include surgery, radiation therapy, chemotherapy, cerebrospinal fluid diversion, observation, and targeted therapy.[1][2][3]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

    • The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.
    • The Cleveland Clinic Web site has an information page on Childhood brain stem glioma. Click on the Cleveland Clinic link to view this page.

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Childhood brain stem glioma. Click on the link to view a sample search on this topic.

        References

        1. Childhood Brain Stem Glioma Treatment (PDQ®)–Patient Version. National Cancer Institute. May 2016; https://www.cancer.gov/types/brain/patient/child-glioma-treatment-pdq#section/_76.
        2. Childhood Brain Stem Glioma Treatment (PDQ®)–Health Professional Version. National Cancer Institute. May 2016; https://www.cancer.gov/types/brain/hp/child-glioma-treatment-pdq.
        3. Joseph C Landolfi, DO. Brainstem Gliomas. Medscape Reference. March 2014; https://emedicine.medscape.com/article/1156030-overview.