Dysembryoplastic neuroepithelial tumour

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Dysembryoplastic neuroepithelial tumour, abbreviated DNET[1] and DNT,[2] is a rare neuropathology tumour that is associated with epilepsy.

General

  • Common benign mixed neuronal-glial tumour cause of drug resistant epilepsy.[3]
  • Paediatric population.
  • WHO grade I (ICD-O: 9413/0)

Gross/radiology

  • Temporal lobe, usually cortical.
  • Variable architecture:[4] cystic, solitary nodular, multinodular.
    • DNET, simple form (only glioneuronal element)[5]
    • DNET, complex form (multinodular architecture of astrocytic or oligodendroglial origin)[6]
    • DNET, non-specific histological form (debated, lacking glioneuronal element) [7]
  • Adjacent cortical dysplasia in up to 80%[8]

  • DNET on FLAIR MRI (wc/Dr Frank Gaillard)

Microscopic

Features:[4][9]

  • Usu. multiple intracortical nodules.
  • Loose textured internodular component (Specific glioneuronal element):
    • Axonal columns oriented to the surface
    • Floating neurons in eosinophilic matrix
    • Lined by cells similar to oligodendrocytes:
      • Large central nuclei with indentations.
      • Multiple small nucleoli (common).
      • Clear cytoplasm.
    • few stellated astrocytes


IHC:[10]

  • MAP2+ve in oligodendroglia-like cells
  • CD34 in 61%
  • Calbindin in 57%
  • Nestin in 86%
  • MIB-1 (Ki-67) 0-8%

Molecular:[11]

  • BRAF V600E
  • FGFR1 tandem duplication (FGFR1-TKDD)

DDx:

  • Oligodendroglioma.
    • These have rounder, smaller nuclei with occasional nucleoli.[4]
    • LOH1p/19q is not seen in DNET. [12]
  • Astrocytoma, protoplasmatic variant.
    • infiltrative microcystic formations may mimic glioneuronal element
  • Ganglioglioma.
    • Has reticulin fibers, lymphocytic cuffs and large cysts.
  • Polymorphous low-grade neuroepithelial tumor of the young” (PLNTY).
    • Usu. strongly CD34+ve.

Images

  • DNT. (WC/Sbrandner)

  • DNT, low power view (WC/jensflorian)

  • Synaptophysin IHC (WC/jensflorian)

  • Smear (WC/jensflorian)

www:

See also

References

  1. ↑ Alexander, H.; Tannenburg, A.; Walker, DG.; Coyne, T. (Jan 2015). "Progressive dysembryoplastic neuroepithelial tumour.". J Clin Neurosci 22 (1): 221-4. doi:10.1016/j.jocn.2014.07.022. PMID 25311417.
  2. ↑ Matyja, E.; Grajkowska, W.; Kunert, P.; Marchel, A. (Oct 2014). "A peculiar histopathological form of dysembryoplastic neuroepithelial tumor with separated pilocytic astrocytoma and rosette-forming glioneuronal tumor components.". Neuropathology 34 (5): 491-8. doi:10.1111/neup.12124. PMID 24735014.
  3. ↑ Cataltepe, O.; Turanli, G.; Yalnizoglu, D.; Topçu, M.; Akalan, N. (Apr 2005). "Surgical management of temporal lobe tumor-related epilepsy in children.". J Neurosurg 102 (3 Suppl): 280-7. doi:10.3171/ped.2005.102.3.0280. PMID 15881751.
  4. ↑ 4.0 4.1 4.2 O'Brien, DF.; Farrell, M.; Delanty, N.; Traunecker, H.; Perrin, R.; Smyth, MD.; Park, TS. (Dec 2007). "The Children's Cancer and Leukaemia Group guidelines for the diagnosis and management of dysembryoplastic neuroepithelial tumours.". Br J Neurosurg 21 (6): 539-49. doi:10.1080/02688690701594817. PMID 18071981.
  5. ↑ Daumas-Duport, C. (Jul 1993). "Dysembryoplastic neuroepithelial tumours.". Brain Pathol 3 (3): 283-95. PMID 8293188.
  6. ↑ Daumas-Duport, C.; Scheithauer, BW.; Chodkiewicz, JP.; Laws, ER.; Vedrenne, C. (Nov 1988). "Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Report of thirty-nine cases.". Neurosurgery 23 (5): 545-56. PMID 3143922.
  7. ↑ Sung, CO.; Suh, YL. (Mar 2009). "Different pattern of expression of nestin in the non-specific form of dysembryoplastic neuroepithelial tumors compared to the simple and complex forms.". J Neurooncol 92 (1): 7-13. doi:10.1007/s11060-008-9725-z. PMID 18998057.
  8. ↑ Sakuta, R.; Otsubo, H.; Nolan, MA.; Weiss, SK.; Hawkins, C.; Rutka, JT.; Chuang, NA.; Chuang, SH. et al. (Apr 2005). "Recurrent intractable seizures in children with cortical dysplasia adjacent to dysembryoplastic neuroepithelial tumor.". J Child Neurol 20 (4): 377-84. PMID 15921242.
  9. ↑ Daumas-Duport, C. (Jul 1993). "Dysembryoplastic neuroepithelial tumours.". Brain Pathol 3 (3): 283-95. PMID 8293188.
  10. ↑ Thom, M.; Toma, A.; An, S.; Martinian, L.; Hadjivassiliou, G.; Ratilal, B.; Dean, A.; McEvoy, A. et al. (Oct 2011). "One hundred and one dysembryoplastic neuroepithelial tumors: an adult epilepsy series with immunohistochemical, molecular genetic, and clinical correlations and a review of the literature.". J Neuropathol Exp Neurol 70 (10): 859-78. doi:10.1097/NEN.0b013e3182302475. PMID 21937911.
  11. ↑ Fina F, Barets D, Colin C, Bouvier C, Padovani L, Nanni-Metellus I, Ouafik L, Scavarda D, Korshunov A, Jones DT, Figarella-Branger D (January 2017). "Droplet digital PCR is a powerful technique to demonstrate frequent FGFR1 duplication in dysembryoplastic neuroepithelial tumors". Oncotarget 8 (2): 2104–2113. doi:10.18632/oncotarget.12881. PMC 5356784. PMID 27791984. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5356784/.
  12. ↑ Fujisawa, H.; Marukawa, K.; Hasegawa, M.; Tohma, Y.; Hayashi, Y.; Uchiyama, N.; Tachibana, O.; Yamashita, J. (Dec 2002). "Genetic differences between neurocytoma and dysembryoplastic neuroepithelial tumor and oligodendroglial tumors.". J Neurosurg 97 (6): 1350-5. doi:10.3171/jns.2002.97.6.1350. PMID 12507133.
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