Rare Pediatrics News

Disease Profile

Ganglioglioma

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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Other names (AKA)

Mixed cell tumors containing both neural ganglionic cells and neural glial cell components

Categories

Rare Cancers

Summary

A ganglioglioma is a rare type of brain tumor, accounting for approximately 1% of all brain tumors.[1] Gangliogliomas occur when a single cell in the brain starts to divide into more cells, forming a tumor.[2] This can occur when the cell randomly acquires changes (mutations) in genes that regulate how a cell divides. Most gangliogliomas grow slowly and are considered benign. However, up to 10% of gangliogliomas may grow more rapidly and become malignant, meaning the tumor affects the surrounding brain tissue.[3] The main treatment for ganglioglioma is removal of the entire tumor during surgery. If the entire tumor is not removed, it has the potential to recur and may require additional surgery or treatments, such as radiation therapy or chemotherapy. Unfortunately, because gangliogliomas are quite rare, there is limited information to show that radiation therapy or chemotherapy are effective treatments for this condition.[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.

    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 Ganglioglioma. Click on the link to view a sample search on this topic.

      References

      1. DeMarchi R, Abu-Abed S, Munoz D, Loch Macdonald R. Malignant ganglioglioma: case report and review of literature. Journal of Neuro-oncology. 2011; 101:311-318. https://www.ncbi.nlm.nih.gov/pubmed/20524041. Accessed 7/8/2011.
      2. Majores M, von Lehe M, Fassunke J, Schramm J, Becker AJ, Simon M. Tumor Recurrence and Malignant Progression of Gangliogliomas. Cancer. 2008; 113:3355-3363. https://www.ncbi.nlm.nih.gov/pubmed/18988291. Accessed 7/7/2011.
      3. Selvanathan SK, Hammouche S, Salminen HJ, Jenkinson MD. Outcome and prognostic features in anaplastic ganglioglioma: analysis of cases from the SEER database. Journal of Neuro-oncology. 2011; Epub ahead of print:https://www.ncbi.nlm.nih.gov/pubmed/21626070. Accessed 10/18/2011.