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Rodriguez FJ, Schniederjan MJ, Nicolaides T, Tihan T, Burger PC, Perry A (2015) High rate of concurrent BRAF-KIAA1549 gene fusion and 1p deletion in disseminated oligodendroglioma-like leptomeningeal neoplasms (DOLN).
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Childs Nerv Syst 33:1411–1414ĭyson K, Rivera-Zengotita M, Kresak J et al (2016) FGFR1 N546K and H3F3A K27M mutations in a diffuse leptomeningeal tumour with glial and neuronal markers. Karlowee V, Kolakshyapati M, Amatya VJ, Takayasu T, Nosaka R, Sugiyama K, Kurisu K, Yamasaki F (2017) Diffuse leptomeningeal glioneuronal tumor (DLGNT) mimicking Whipple’s disease: a case report and literature review. Preuss M, Christiansen H, Merkenschlager A, Hirsch FW, Kiess W, Müller W, Kästner S, Henssler A, Pekrun A, Hauch H, Nathrath M, Meixensberger J, Pietsch T, Kuchelmeister K (2015) Disseminated oligodendroglial-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity. Am J Surg Pathol 37:763–771Ĭho HJ, Myung JK, Kim H, Park CK, Kim SK, Chung CK, Choi SH, Park SH (2015) Primary diffuse leptomeningeal glioneuronal tumors. Schniederjan MJ, Alghamdi S, Castellano-Sanchez A, Mazewski C, Brahma B, Brat DJ, Brathwaite CD, Janss AJ (2013) Diffuse leptomeningeal neuroepithelial tumor: 9 pediatric cases with chromosome 1p/19q deletion status and IDH1 (R132H) immunohistochemistry. Schwetye KE, Kansagra AP, McEachern J, Schmidt RE, Gauvain K, Dahiya S (2017) Unusual high-grade features in pediatric diffuse leptomeningeal glioneuronal tumor: comparison with a typical low-grade example.
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In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (Eds) WHO classification of tumours of the central nervous system (Revised 4th edition). Reifenberger G, Rodriguez F, Burger PC, Perry A, Capper D (2016) Diffuse leptomeningeal glioneuronal tumour. Although H3K27M mutations have been described in rare examples of low-grade glial and glioneuronal tumors, whether DL-GNTs with H3K27M represent a rare growth pattern of the aggressive H3K27M-mutant diffuse midline gliomas needs further clarification. Only one previous case of DL-GNT has been reported to harbour H3K27M mutation.
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Further molecular analysis revealed 1p and 19q co-deletion and H3K27M mutation, while no mutation were identified in IDH, TERT, or BRAF genes. Biopsy from the spinal mass showed histomorphological features characteristic of DL-GNT. Imaging showed leptomeningeal thickening and spinal lesions. We present a rare instance of a DL-GNT in a 13-year-old female who presented with slowly progressive and sequential neurological deficits over a 12-month duration. Their molecular genetic profile is not fully elucidated and few reports have identified chromosome 1p and 19q deletions, and BRAF alterations. They are surgically challenging tumors with a high incidence of delayed morbidity and mortality despite low-grade histology. Predominantly occurring in children, these tumors present as chronic meningitis and mimic infectious/inflammatory diseases. Diffuse leptomeningeal glioneuronal tumor (DL-GNT) is a newly introduced tumor entity of uncertain prognosis characterised by a primary diffuse leptomeningeal growth pattern, oligodendroglial-like morphology and dual glial/neuronal differentiation.