National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Papillary glioneuronal tumors




Papillary glioneuronal tumors (GNTs) are rare tumors. They may occur in any age (4–75 years), but are more common in young patients.[1] Symptoms may include seizures, headaches, bleedings, and focal neurological deficits, impairing specific and small parts of the body.[1][2] According to the 2016 WHO classification of CNS tumors  the papillary GNTs are considered WHO grade I tumors.[3] The grading of a tumor refers about how the tumor tissue look under a microscope, and indicates how quickly a tumor is likely to grow and spread. In grade 1 tumors, the tumor cells and the organization of the tumor tissue appear close to normal and they tend to grow and spread slowly.[4] Papillary GNTs are usually located in the cerebral hemispheres (the two halves of the brain) next to the cerebral ventricles, the fluid-filled cavities of the brain. They are mixed tumors that contain both neural cells (neurons),  which transmit the impulses in the brain, and glial cells (astrocytes) that help support the neurons. Treatment is done with surgery to remove the tumor and usually results in a good prognosis with long-term survival.[1]
Last updated: 10/31/2017

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  1. Soffietti R, Rudà R & Reardon D. Rare glial tumors. Handb Clin Neurol. 2016; 134:399-415. https://www.ncbi.nlm.nih.gov/pubmed/26948368.
  2. Rezaee A. Papillary glioneuronal tumour. Radiopaedia. https://radiopaedia.org/articles/papillary-glioneuronal-tumour.
  3. Louis DN, Perry A, Reifenberger G et al. Acta Neuropathol. WHO Central Nervous System Tumor Classification. 2016; https://braintumor.org/wp-content/assets/WHO-Central-Nervous-System-Tumor-Classification.pdf.
  4. Tumor Grade. National Cancer Institute. 2013; https://www.cancer.gov/about-cancer/diagnosis-staging/prognosis/tumor-grade-fact-sheet.