Rare Immunology News

Disease Profile

Gliomatosis cerebri

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

Adult

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ICD-10

C71.0

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

Nervous System Diseases

Summary

Gliomatosis cerebri is a type of malignant (cancerous) brain tumor called a glioblastoma that develops from a specific type of brain cell called an astrocyte.[1] It involves at least three sections (lobes) within the main part of the brain (cerebrum). It may also affect both sides of the cerebrum as well as the nerve cells within the brain (grey matter). There are two types of gliomatosis cerebri. Type 1 (classic form) is characterized by scattered and widespread tumor cells and no apparent mass or tumor, while type 2 has similar characteristics in addition to a detectable mass or tumor.[2] Signs and symptoms of gliomatosis cerebri may include personality changes, memory disturbance, headaches, and seizures.[3] Because this type of cancer can be found in many areas of the brain, it can be challenging to treat. Treatment might include surgery to remove areas of the brain affected (when possible), radiation therapy, and chemotherapy.[2]

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 National Cancer Institute (NCI) provides current information on brain tumors. The NCI conducts and supports cancer research and training and provides cancer information to patients, health professionals, and the general public.
    • The Weill Cornell Brain and Spine Center provides information on Gliomatosis cerebri

      In-Depth Information

      • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
      • 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.
      • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Gliomatosis cerebri. Click on the link to view a sample search on this topic.

        References

        1. Jeffrey Greenfield. Gliomatosis Cerebri. Weill Cornell Brain and Spine Center. Janurary 2015; https://weillcornellbrainandspine.org/condition/gliomatosis-cerebri.
        2. Chen S, Tanaka S, Giannini C, Morris J, Yan ES, Buckner J, Lachance DH, Parney IF. Gliomatosis cerebri: clinical characteristics, management, and outcomes. J Neurooncol. April 2013; 112(2):267-75. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3907195/.
        3. Uddin S, Jarmi T. Neurologic Manifestations of Glioblastoma Multiforme. Medscape. Nov 9, 2015; https://emedicine.medscape.com/article/1156220-overview.