Diffuse leptomeningeal glioneuronal tumour

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Diffuse leptomeningeal glioneuronal tumour (abbreviated DLGNT) is a neuropathology tumour related to pilocytic astrocytoma. The future WHO classification of CNS tumours will include it as separate entity.

General

Features:

  • Currently no WHO grade assigned. Molecular class MC-1 corresponds to WHO I, class MC-2 to WHO II/III[1]
  • Low-grade lesion but anaplastic progression reported.
  • Previously known as Disseminated oligodendroglial-like leptomeningeal tumour (abbreviated DOLN)
    • AKA "disseminated oligodendroglial-like leptomeningeal tumor of childhood" (DOGLT).[2]
  • Intermediate prognosis.

Gross

Features:

  • Multiple tumor sites (60-85% spinal).
  • Diffuse leptomeningeal expansion.
    • Hydrocaphalus due to CSF occlusion.
    • May spread extraneural through shunts. [3]
    • Rarely Cysts.[4]

Microscopic

Features:

  • Oligodendroglioma-like cells with low-mitotic activity.
  • Rare ganglion/ganglioid cells.
  • Ocassionally eosinophilic granular bodies and Rosenthal fibers.
  • Infiltrative growth pattern.
  • Variable mitotic activity.

IHC

Fetaures:[5][6]

  • OLIG2 (7 of 9) (78 %).
  • S100 (11 of 12) (92 %).
  • GFAP (12 of 31) (39 %).
  • Synaptophysin (19 of 27) (70 %).
  • NeuN -ve.
  • EMA -ve.
  • IDH-1 -ve.
  • HuC/D +/-ve.
  • CD99 -ve.

Molecular

  • Mixture of partially oligodendroglioma, partially pilocytic astrocytoma features.[1]
  • 1p loss in 100% (or combined 1p/19q loss in 15-50%)[7]
  • KIAA1549-BRAF fusions.[9]
    • High frequency of co-occurence of BRAF-KIAA1549 fusions and 1p deletion.
    • BRAF-KIAA1549 fusions are usu. absent in oligodendroglioma.[10]
  • BRAF V600E-negative.
  • IDH1/2-Wildtype.
  • Two methylation class subgroups (DLGNT MC-1 and MC-2)
    • 1p gain in all MC-2 class cases.
    • MC-1 class cases are younger and have a more favourable prognosis.

Images

  • Radiopedia.org [[1]]

  • DOLN - HE low mag. (WC/jensflorian)

  • DOLN - HE intermed mag. (WC/jensflorian)

  • DOLN - HE high mag. (WC/jensflorian)

DDx

See also

References

  1. ↑ 1.0 1.1 Deng MY, Sill M, Chiang J, Schittenhelm J, Ebinger M, Schuhmann MU, Monoranu CM, Milde T, Wittmann A, Hartmann C, Sommer C, Paulus W, Gärtner J, Brück W, Rüdiger T, Leipold A, Jaunmuktane Z, Brandner S, Giangaspero F, Nozza P, Mora J, Morales la Madrid A, Cruz Martinez O, Hansford JR, Pietsch T, Tietze A, Hernáiz-Driever P, Stoler I, Capper D, Korshunov A, Ellison DW, von Deimling A, Pfister SM, Sahm F, Jones DTW (August 2018). "Molecularly defined diffuse leptomeningeal glioneuronal tumor (DLGNT) comprises two subgroups with distinct clinical and genetic features". Acta Neuropathol 136 (2): 239–253. doi:10.1007/s00401-018-1865-4. PMID 29766299.
  2. ↑ Preuss, M.; Christiansen, H.; Merkenschlager, A.; Hirsch, FW.; Kiess, W.; Müller, W.; Kästner, S.; Henssler, A. et al. (Aug 2015). "Disseminated oligodendroglial-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity [corrected].". J Neurooncol 124 (1): 65-74. doi:10.1007/s11060-015-1735-z. PMID 25672644.
  3. ↑ Kessler, BA.; Bookhout, C.; Jaikumar, S.; Hipps, J.; Lee, YZ.. "Disseminated oligodendroglial-like leptomeningeal tumor with anaplastic progression and presumed extraneural disease: case report.". Clin Imaging 39 (2): 300-4. doi:10.1016/j.clinimag.2014.11.018. PMID 25518979.
  4. ↑ Acar, NP.; Oguz, KK.; Kurne, AT.; Nurlu, G.; Ozer, H.; Soylemezoglu, F. (Mar 2015). "Disseminated cystic lesions: a case of disseminated oligodendroglial-like leptomeningeal tumor.". Neurology 84 (13): 1382-3. doi:10.1212/WNL.0000000000001410. PMID 25825464.
  5. ↑ Rodriguez, FJ.; Perry, A.; Rosenblum, MK.; Krawitz, S.; Cohen, KJ.; Lin, D.; Mosier, S.; Lin, MT. et al. (Nov 2012). "Disseminated oligodendroglial-like leptomeningeal tumor of childhood: a distinctive clinicopathologic entity.". Acta Neuropathol 124 (5): 627-41. doi:10.1007/s00401-012-1037-x. PMID 22941225.
  6. ↑ Preuss, M.; Christiansen, H.; Merkenschlager, A.; Hirsch, FW.; Kiess, W.; Müller, W.; Kästner, S.; Henssler, A. et al. (Aug 2015). "Disseminated oligodendroglial-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity [corrected].". J Neurooncol 124 (1): 65-74. doi:10.1007/s11060-015-1735-z. PMID 25672644.
  7. ↑ Bourne, TD.; Mandell, JW.; Matsumoto, JA.; Jane, JA.; Lopes, MB. (Dec 2006). "Primary disseminated leptomeningeal oligodendroglioma with 1p deletion. Case report.". J Neurosurg 105 (6 Suppl): 465-9. doi:10.3171/ped.2006.105.6.465. PMID 17184079.
  8. ↑ Preuss, M.; Christiansen, H.; Merkenschlager, A.; Hirsch, FW.; Kiess, W.; Müller, W.; Kästner, S.; Henssler, A. et al. (Aug 2015). "Disseminated oligodendroglial-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity [corrected].". J Neurooncol 124 (1): 65-74. doi:10.1007/s11060-015-1735-z. PMID 25672644.
  9. ↑ Rodriguez, FJ.; Schniederjan, MJ.; Nicolaides, T.; Tihan, T.; Burger, PC.; Perry, A. (Apr 2015). "High rate of concurrent BRAF-KIAA1549 gene fusion and 1p deletion in disseminated oligodendroglioma-like leptomeningeal neoplasms (DOLN).". Acta Neuropathol 129 (4): 609-10. doi:10.1007/s00401-015-1400-9. PMID 25720745.
  10. ↑ Gierke, M.; Sperveslage, J.; Schwab, D.; Beschorner, R.; Ebinger, M.; Schuhmann, MU.; Schittenhelm, J. (Jun 2015). "Analysis of IDH1-R132 mutation, BRAF V600 mutation and KIAA1549-BRAF fusion transcript status in central nervous system tumors supports pediatric tumor classification.". J Cancer Res Clin Oncol. doi:10.1007/s00432-015-2006-2. PMID 26115961.
  11. ↑ Bourne, TD.; Mandell, JW.; Matsumoto, JA.; Jane, JA.; Lopes, MB. (Dec 2006). "Primary disseminated leptomeningeal oligodendroglioma with 1p deletion. Case report.". J Neurosurg 105 (6 Suppl): 465-9. doi:10.3171/ped.2006.105.6.465. PMID 17184079.
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