Diffuse leptomeningeal glioneuronal tumor explained

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. 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]

Clinical features

Patients with DLGNT presented with a variety of clinical manifestations, depending on the involved area of the disease, ranging from, numbness and seizure to hydrocephalus symptoms Irritability, headaches and vomiting.[15] [16] The progression of the disease is slow, however there have been reports of anaplastic transition.[17] [18]

Diagnosis

MRI reveals broad leptomeningeal enhancing and thickness, which is frequently most visible throughout the spine, brainstem, and posterior fossa.[19]

Notes and References

  1. 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
  2. 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
  3. 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
  4. 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
  5. 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
  6. 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
  7. 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
  8. Korein J, Feigin I, Shapiro MF (1957) Oligodendrogliomatosis with intracranial hypertension. Neurology 7:589–594
  9. Mathews MS, Pare LS, Kuo JV, Kim RC (2009) Primary leptomeningeal oligodendrogliomatosis. J Neurooncol 94:275–278
  10. Chen R, Macdonald DR, Ramsay DA (1995) Primary diffuse leptomeningeal oligodendroglioma. Case report. J Neurosurgery 83:724–728
  11. Gardiman MP, Fassan M, Orvieto E et al (2010) Diffuse leptomeningeal glioneuronal tumors: a new entity? Brain Pathol 20:361–366
  12. Davila G, Duyckaerts C, Lazareth JP et al (1993) Diffuse primary leptomeningeal gliomatosis. J Neurooncol 15:45–49
  13. 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
  14. 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
  15. 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. Am J Surg Pathol 37:763–771. https://doi.org/10.1097/PAS.0b013e31827bf4cc
  16. Dodgshun AJ, SantaCruz N, Hwang J, Ramkissoon SH, Malkin H, Bergthold G, Manley P, Chi S, MacGregor D, Goumnerova L et al (2016) Disseminated glioneuronal tumors occurring in childhood: treatment outcomes and BRAF alterations including V600E mutation. J Neurooncol 128:293–302. https://doi.org/10.1007/s11060-016-2109-x
  17. Yamasaki, T.; Sakai, N.; Shinmura, K.; Kawaji, H.; Koizumi, S.; Samashima, T.; Namba, H. Anaplastic changes of diffuse leptomeningeal glioneuronal tumor with polar spongioblastoma pattern. Brain Tumor Pathol. 2018, 35, 209–216.
  18. Schwetye, K.E.; Kansagra, A.P.; McEachern, J.; Schmidt, R.E.; Gauvain, K.; Dahiya, S. Unusual highgrade features in pediatric diffuse leptomeningeal glioneuronal tumor: Comparison with a typical low-grade example. Hum. Pathol. 2017, 70, 105–112.
  19. Rossi S, Rodriguez FJ, Mota RA, Dei Tos AP, Di Paola F, Bendini M, Agostini S, Longatti P, Jenkins RB, Giannini C (2009) Primary leptomeningeal oligodendroglioma with documented progression to anaplasia and t(1;19) (q10;p10) in a child. Acta Neuropathol 118:575–577. https://doi.org/10.1007/s00401-009-0565-5

This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Diffuse leptomeningeal glioneuronal tumor".

Except where otherwise indicated, Everything.Explained.Today is © Copyright 2009-2024, A B Cryer, All Rights Reserved. Cookie policy.