Rare primary CNS tumor
| This article has multiple issues. Please help improve it or discuss these issues on the talk page. (Learn how and when to remove these template messages)
(Learn how and when to remove this message) |
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] It's characterised by wide leptomeningeal spread[5] with male predominance,[6] like histopathology of neurocytoma,[7] oligodendrocyte-like cytopathology,[8] bland appearance, and severe clinical behaviour.[9][10] Children's basal cisterns and inter-hemispheric fissures are typically involved in plaque like subarachnoid tumors. A common related intraparenchymal lesion is a spinal lesion.[11] However, in certain situations, superficial parenchyma or Virchow-Robin gaps were affected.[12][13]
Molecular and genetic investigations frequently show a combination of KIAA1549 and the serine/threonine protein kinase BRAF gene, and also deletions of the short arm of chromosome number 1 and/or the long arm of chromosome number 19.[14]
- ^ Johnson, D.R.; Guerin, J.B.; Giannini, C.; Morris, J.M.; Eckel, L.J.; Kaufmann, T.J. 2016 Updates to the WHO Brain Tumor Classification System: What the Radiologist Needs to Know. Radiographics 2017, 37, 2164–2180
- ^ Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW (2016) The 2016 World Health Organization classifcation of tumors of the central nervous system: a summary. Acta Neuropathol 131:803–820. https://doi.org/10.1007/s00401-016-1545-1
- ^ a b Rodriguez FJ, Perry A, Rosenblum MK, Krawitz S, Cohen KJ, Lin D, Mosier S, Lin MT, Eberhart CG, Burger PC (2012) Disseminated oligodendroglial-like leptomeningeal tumor of childhood: a distinctive clinicopathologic entity. Acta Neuropathol 124:627–641. doi:10.1007/s00401-012-1037-x
- ^ Chiang JCH, Harreld JH, Orr BA, Sharma S, Ismail A, Segura AD, Ellison DW (2017) Low-grade spinal glioneuronal tumors with BRAF gene fusion and 1p deletion but without leptomeningeal dissemination. Acta Neuropathol 134:159–162. https://doi.org/10.1007/s00401-017-1728-4
- ^ Agamanolis DP, Katsetos CD, Klonk CJ, Bartkowski HM, Ganapathy S, Staugaitis SM, Kuerbitz SJ, Patton DF, Talaizadeh A, Cohen BH (2012) An unusual form of superficially disseminated glioma in children: report of 3 cases. J Child Neurol 27:727–733
- ^ Lyle, M.R.; Dolia, J.N.; Fratkin, J.; Nichols, T.A.; Herrington, B.L. Newly Identified Characteristics and Suggestions for Diagnosis and Treatment of Diffuse Leptomeningeal Glioneuronal/Neuroepithelial Tumors: A Case Report and Review of the Literature. Child Neurol. Open 2015, 2, 2329048X14567531
- ^ Psarros TG, Swift D, Mulne AF et al (2005) Neurocytoma-like neoplasm of the thoracic spine in a 15-month-old child presenting with diffuse leptomeningeal dissemination and communicating hydrocephalus. Case report. J Neurosurg 103:184–190
- ^ Korein J, Feigin I, Shapiro MF (1957) Oligodendrogliomatosis with intracranial hypertension. Neurology 7:589–594
- ^ Mathews MS, Pare LS, Kuo JV, Kim RC (2009) Primary leptomeningeal oligodendrogliomatosis. J Neurooncol 94:275–278
- ^ Chen R, Macdonald DR, Ramsay DA (1995) Primary diffuse leptomeningeal oligodendroglioma. Case report. J Neurosurgery 83:724–728
- ^ Gardiman MP, Fassan M, Orvieto E et al (2010) Diffuse leptomeningeal glioneuronal tumors: a new entity? Brain Pathol 20:361–366
- ^ Davila G, Duyckaerts C, Lazareth JP et al (1993) Diffuse primary leptomeningeal gliomatosis. J Neurooncol 15:45–49
- ^ Kastenbauer S, Danek A, Klein W et al (2000) Primary diffuse leptomeningeal gliomatosis: unusual MRI with non-enhancing nodular lesions on the cerebellar surface and spinal leptomeningeal enhancement. J Neurol Neurosurg Psychiatry 69:385–388
- ^ Rodriguez, F.J.; Schniederjan, M.J.; Nicolaides, T.; Tihan, T.; Burger, P.C.; Perry, A. High rate of concurrent BRAF-KIAA1549 gene fusion and 1p deletion in disseminated oligodendroglioma-like leptomeningeal neoplasms (DOLN). Acta Neuropathol. 2015, 129, 609–610