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Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare, primary CNS tumor, classified as distinct entity in 2016 [1] and described as diffuse oligodendroglial-like leptomeningeal tumor of children in the 2016 classification of CNS neoplasms by the WHO., [2] Typically, it's considered juvenile tumors [3] but can occur in adults, [4] the average age of diagnosis is five years. [3]
Leptomeningeal cancer is a rare complication of cancer in which the disease spreads from the original tumor site to the meninges surrounding the brain and spinal cord. [1] This leads to an inflammatory response, hence the alternative names neoplastic meningitis (NM), malignant meningitis , or carcinomatous meningitis .
Diffuse midline glioma, H3 K27-altered (DMG) is a fatal tumour that arises in midline structures of the brain, most commonly the brainstem, thalamus and spinal cord. When located in the pons it is also known as diffuse intrinsic pontine glioma ( DIPG ).
1.5.4 Diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters 1.5.5 Papillary glioneuronal tumor 1.5.6 Rosette-forming glioneuronal tumor 1.5.7 Myxoid glioneuronal tumor 1.5.8 Diffuse leptomeningeal glioneuronal tumor 1.5.9 Gangliocytoma 1.5.10 Multinodular and vacuolating neuronal tumor
A glioma is a type of primary tumor that starts in the glial cells of the brain or spinal cord.They are cancerous but some are extremely slow to develop. [2] [3] Gliomas comprise about 30 percent of all brain tumors and central nervous system tumours, and 80 percent of all malignant brain tumours.
As such, this is an exceptionally unusual diagnosis. The updated WHO guidelines published in 2007 recommends classifying such tumors for the time being as 'glioblastoma with oligodendroglioma component'. [8] It remains to be established whether or not these tumors carry a better prognosis than standard glioblastomas.