Difference between revisions of "Dysembryoplastic neuroepithelial tumour"

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'''Dysembryoplastic neuroepithelial tumour''', abbreviated '''DNT''', is a rare [[neuropathology tumour]] that is associated with [[epilepsy]].
'''Dysembryoplastic neuroepithelial tumour''', abbreviated '''DNET'''<ref name=pmid25311417>{{Cite journal  | last1 = Alexander | first1 = H. | last2 = Tannenburg | first2 = A. | last3 = Walker | first3 = DG. | last4 = Coyne | first4 = T. | title = Progressive dysembryoplastic neuroepithelial tumour. | journal = J Clin Neurosci | volume = 22 | issue = 1 | pages = 221-4 | month = Jan | year = 2015 | doi = 10.1016/j.jocn.2014.07.022 | PMID = 25311417 }}</ref> and '''DNT''',<ref name=pmid24735014>{{Cite journal  | last1 = Matyja | first1 = E. | last2 = Grajkowska | first2 = W. | last3 = Kunert | first3 = P. | last4 = Marchel | first4 = A. | title = A peculiar histopathological form of dysembryoplastic neuroepithelial tumor with separated pilocytic astrocytoma and rosette-forming glioneuronal tumor components. | journal = Neuropathology | volume = 34 | issue = 5 | pages = 491-8 | month = Oct | year = 2014 | doi = 10.1111/neup.12124 | PMID = 24735014 }}</ref> is a rare [[neuropathology tumour]] that is associated with [[epilepsy]].


==General==
==General==
*Common tumour cause of drug resistant [[epilepsy]].<ref name=pmid15881751>{{Cite journal  | last1 = Cataltepe | first1 = O. | last2 = Turanli | first2 = G. | last3 = Yalnizoglu | first3 = D. | last4 = Topçu | first4 = M. | last5 = Akalan | first5 = N. | title = Surgical management of temporal lobe tumor-related epilepsy in children. | journal = J Neurosurg | volume = 102 | issue = 3 Suppl | pages = 280-7 | month = Apr | year = 2005 | doi = 10.3171/ped.2005.102.3.0280 | PMID = 15881751 }}</ref>
*Common benign mixed neuronal-glial tumour cause of drug resistant [[epilepsy]].<ref name=pmid15881751>{{Cite journal  | last1 = Cataltepe | first1 = O. | last2 = Turanli | first2 = G. | last3 = Yalnizoglu | first3 = D. | last4 = Topçu | first4 = M. | last5 = Akalan | first5 = N. | title = Surgical management of temporal lobe tumor-related epilepsy in children. | journal = J Neurosurg | volume = 102 | issue = 3 Suppl | pages = 280-7 | month = Apr | year = 2005 | doi = 10.3171/ped.2005.102.3.0280 | PMID = 15881751 }}</ref>
*Paediatric population.
*Paediatric population.
* WHO grade I (ICD-O: 9413/0)


==Gross/radiology==
==Gross/radiology==
*Temporal lobe.
*Temporal lobe, usually cortical.
*Variable architecture:<ref name=pmid18071981/> cystic, solitary nodular, multinodular.  
*Variable architecture:<ref name=pmid18071981/> cystic, solitary nodular, multinodular.  
** DNET, simple form (only glioneuronal element)<ref>{{Cite journal  | last1 = Daumas-Duport | first1 = C. | title = Dysembryoplastic neuroepithelial tumours. | journal = Brain Pathol | volume = 3 | issue = 3 | pages = 283-95 | month = Jul | year = 1993 | doi =  | PMID = 8293188 }}</ref>
** DNET, complex form (multinodular architecture of astrocytic or oligodendroglial origin)<ref>{{Cite journal  | last1 = Daumas-Duport | first1 = C. | last2 = Scheithauer | first2 = BW. | last3 = Chodkiewicz | first3 = JP. | last4 = Laws | first4 = ER. | last5 = Vedrenne | first5 = C. | title = Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Report of thirty-nine cases. | journal = Neurosurgery | volume = 23 | issue = 5 | pages = 545-56 | month = Nov | year = 1988 | doi =  | PMID = 3143922 }}</ref>
** DNET, non-specific histological form (debated, lacking glioneuronal element) <ref>{{Cite journal  | last1 = Sung | first1 = CO. | last2 = Suh | first2 = YL. | title = Different pattern of expression of nestin in the non-specific form of dysembryoplastic neuroepithelial tumors compared to the simple and complex forms. | journal = J Neurooncol | volume = 92 | issue = 1 | pages = 7-13 | month = Mar | year = 2009 | doi = 10.1007/s11060-008-9725-z | PMID = 18998057 }}</ref>
*Adjacent cortical dysplasia in up to 80%<ref>{{Cite journal  | last1 = Sakuta | first1 = R. | last2 = Otsubo | first2 = H. | last3 = Nolan | first3 = MA. | last4 = Weiss | first4 = SK. | last5 = Hawkins | first5 = C. | last6 = Rutka | first6 = JT. | last7 = Chuang | first7 = NA. | last8 = Chuang | first8 = SH. | last9 = Snead | first9 = OC. | title = Recurrent intractable seizures in children with cortical dysplasia adjacent to dysembryoplastic neuroepithelial tumor. | journal = J Child Neurol | volume = 20 | issue = 4 | pages = 377-84 | month = Apr | year = 2005 | doi =  | PMID = 15921242 }}</ref>
<gallery>
File:DNET02.jpg | DNET on FLAIR MRI (wc/Dr Frank Gaillard)
</gallery>


==Microscopic==
==Microscopic==
Features:<ref name=pmid18071981>{{Cite journal  | last1 = O'Brien | first1 = DF. | last2 = Farrell | first2 = M. | last3 = Delanty | first3 = N. | last4 = Traunecker | first4 = H. | last5 = Perrin | first5 = R. | last6 = Smyth | first6 = MD. | last7 = Park | first7 = TS. | title = The Children's Cancer and Leukaemia Group guidelines for the diagnosis and management of dysembryoplastic neuroepithelial tumours. | journal = Br J Neurosurg | volume = 21 | issue = 6 | pages = 539-49 | month = Dec | year = 2007 | doi = 10.1080/02688690701594817 | PMID = 18071981 }}</ref>
Features:<ref name=pmid18071981>{{Cite journal  | last1 = O'Brien | first1 = DF. | last2 = Farrell | first2 = M. | last3 = Delanty | first3 = N. | last4 = Traunecker | first4 = H. | last5 = Perrin | first5 = R. | last6 = Smyth | first6 = MD. | last7 = Park | first7 = TS. | title = The Children's Cancer and Leukaemia Group guidelines for the diagnosis and management of dysembryoplastic neuroepithelial tumours. | journal = Br J Neurosurg | volume = 21 | issue = 6 | pages = 539-49 | month = Dec | year = 2007 | doi = 10.1080/02688690701594817 | PMID = 18071981 }}</ref><ref>{{Cite journal  | last1 = Daumas-Duport | first1 = C. | title = Dysembryoplastic neuroepithelial tumours. | journal = Brain Pathol | volume = 3 | issue = 3 | pages = 283-95 | month = Jul | year = 1993 | doi =  | PMID = 8293188 }}</ref>
*Cells similar to oligodendrocytes:
*Usu. multiple intracortical nodules.
**Large central nuclei with indentations.
*Loose textured internodular component (Specific glioneuronal element):
**Multiple small nucleoli (common).
**Axonal columns oriented to the surface
**Clear cytoplasm.
**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:<ref>{{Cite journal  | last1 = Thom | first1 = M. | last2 = Toma | first2 = A. | last3 = An | first3 = S. | last4 = Martinian | first4 = L. | last5 = Hadjivassiliou | first5 = G. | last6 = Ratilal | first6 = B. | last7 = Dean | first7 = A. | last8 = McEvoy | first8 = A. | last9 = Sisodiya | first9 = SM. | title = One hundred and one dysembryoplastic neuroepithelial tumors: an adult epilepsy series with immunohistochemical, molecular genetic, and clinical correlations and a review of the literature. | journal = J Neuropathol Exp Neurol | volume = 70 | issue = 10 | pages = 859-78 | month = Oct | year = 2011 | doi = 10.1097/NEN.0b013e3182302475 | PMID = 21937911 }}</ref>
*MAP2+ve in oligodendroglia-like cells
*CD34 in 61%
*Calbindin in 57%
*Nestin in 86%
*MIB-1 (Ki-67) 0-8%
 
Molecular:<ref name="pmid27791984">{{cite journal |authors=Fina F, Barets D, Colin C, Bouvier C, Padovani L, Nanni-Metellus I, Ouafik L, Scavarda D, Korshunov A, Jones DT, Figarella-Branger D |title=Droplet digital PCR is a powerful technique to demonstrate frequent FGFR1 duplication in dysembryoplastic neuroepithelial tumors |journal=Oncotarget |volume=8 |issue=2 |pages=2104–2113 |date=January 2017 |pmid=27791984 |pmc=5356784 |doi=10.18632/oncotarget.12881 |url=}}</ref>
* BRAF V600E
* FGFR1 tandem duplication (FGFR1-TKDD)


DDx:
DDx:
*[[Oligodendroglioma]].
*[[Oligodendroglioma]].
**These have rounder, smaller nuclei with occasional nucleoli.<ref name=pmid18071981/>
**These have rounder, smaller nuclei with occasional nucleoli.<ref name=pmid18071981/>
**LOH1p/19q is not seen in DNET. <ref>{{Cite journal  | last1 = Fujisawa | first1 = H. | last2 = Marukawa | first2 = K. | last3 = Hasegawa | first3 = M. | last4 = Tohma | first4 = Y. | last5 = Hayashi | first5 = Y. | last6 = Uchiyama | first6 = N. | last7 = Tachibana | first7 = O. | last8 = Yamashita | first8 = J. | title = Genetic differences between neurocytoma and dysembryoplastic neuroepithelial tumor and oligodendroglial tumors. | journal = J Neurosurg | volume = 97 | issue = 6 | pages = 1350-5 | month = Dec | year = 2002 | doi = 10.3171/jns.2002.97.6.1350 | PMID = 12507133 }}</ref>
*[[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===
===Images===
<gallery>
<gallery>
Image:DNET_HE.jpg | DNT. (WC/Sbrandner)
Image:DNET_HE.jpg | DNT. (WC/Sbrandner)
File:DNT low power.jpg | DNT, low power view (WC/jensflorian)
File:Synaptophysin DNET.jpg | Synaptophysin IHC (WC/jensflorian)
File:He cytology DNET.jpg | Smear (WC/jensflorian)
</gallery>
</gallery>
www:
www:

Latest revision as of 17:18, 25 March 2021

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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