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 unfavourable 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 "Molecularly defined diffuse leptomeningeal glioneuronal tumor (DLGNT) comprises two subgroups with distinct clinical and genetic features". Acta Neuropathol 136 (2): 239–253. August 2018. 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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