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Dysembryoplastic neuroepithelial tumour (view source)
Revision as of 09:57, 14 April 2015
, 09:57, 14 April 2015+ references +histology +IHC
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Jensflorian (talk | contribs) (+ references +histology +IHC) |
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==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> | ||
* | *Specific glioneuronal element: | ||
**Large central nuclei with indentations. | **Axonal columns oriented to the surface | ||
**Multiple small nucleoli (common). | **Floating neurons in eosinophilic matrix | ||
**Clear cytoplasm. | **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: | |||
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. | |||
===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: | ||