Difference between revisions of "Disseminated oligodendroglial-like leptomeningeal 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.
#REDIRECT [[Diffuse leptomeningeal glioneuronal tumour]]
 
==General==
Features:
*Currently no WHO grade assigned.
* Previously known as '''Disseminated oligodendroglial-like leptomeningeal tumour''' (abbreviated DOLN)
*Low-grade lesion but anaplastic progression reported.
*AKA "diffuse leptomeningeal glioneural tumor" or "disseminated oligodendroglial-like leptomeningeal tumor of childhood" (DOGLT).<ref>{{Cite journal  | last1 = Preuss | first1 = M. | last2 = Christiansen | first2 = H. | last3 = Merkenschlager | first3 = A. | last4 = Hirsch | first4 = FW. | last5 = Kiess | first5 = W. | last6 = Müller | first6 = W. | last7 = Kästner | first7 = S. | last8 = Henssler | first8 = A. | last9 = Pekrun | first9 = A. | title = Disseminated oligodendroglial-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity [corrected]. | journal = J Neurooncol | volume = 124 | issue = 1 | pages = 65-74 | month = Aug | year = 2015 | doi = 10.1007/s11060-015-1735-z | PMID = 25672644 }}</ref>
*Intermediate prognosis.
 
 
==Gross==
Features:
*No solid tumour.
*Diffuse leptomeningeal expansion.
**Hydrocaphalus due to CSF occlusion.
**May spread extraneural through shunts. <ref>{{Cite journal  | last1 = Kessler | first1 = BA. | last2 = Bookhout | first2 = C. | last3 = Jaikumar | first3 = S. | last4 = Hipps | first4 = J. | last5 = Lee | first5 = YZ. | title = Disseminated oligodendroglial-like leptomeningeal tumor with anaplastic progression and presumed extraneural disease: case report. | journal = Clin Imaging | volume = 39 | issue = 2 | pages = 300-4 | month =  | year =  | doi = 10.1016/j.clinimag.2014.11.018 | PMID = 25518979 }}</ref>
**Rarely Cysts.<ref>{{Cite journal  | last1 = Acar | first1 = NP. | last2 = Oguz | first2 = KK. | last3 = Kurne | first3 = AT. | last4 = Nurlu | first4 = G. | last5 = Ozer | first5 = H. | last6 = Soylemezoglu | first6 = F. | title = Disseminated cystic lesions: a case of disseminated oligodendroglial-like leptomeningeal tumor. | journal = Neurology | volume = 84 | issue = 13 | pages = 1382-3 | month = Mar | year = 2015 | doi = 10.1212/WNL.0000000000001410 | PMID = 25825464 }}</ref>
 
==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:<ref>{{Cite journal  | last1 = Rodriguez | first1 = FJ. | last2 = Perry | first2 = A. | last3 = Rosenblum | first3 = MK. | last4 = Krawitz | first4 = S. | last5 = Cohen | first5 = KJ. | last6 = Lin | first6 = D. | last7 = Mosier | first7 = S. | last8 = Lin | first8 = MT. | last9 = Eberhart | first9 = CG. | title = Disseminated oligodendroglial-like leptomeningeal tumor of childhood: a distinctive clinicopathologic entity. | journal = Acta Neuropathol | volume = 124 | issue = 5 | pages = 627-41 | month = Nov | year = 2012 | doi = 10.1007/s00401-012-1037-x | PMID = 22941225 }}</ref><ref>{{Cite journal  | last1 = Preuss | first1 = M. | last2 = Christiansen | first2 = H. | last3 = Merkenschlager | first3 = A. | last4 = Hirsch | first4 = FW. | last5 = Kiess | first5 = W. | last6 = Müller | first6 = W. | last7 = Kästner | first7 = S. | last8 = Henssler | first8 = A. | last9 = Pekrun | first9 = A. | title = Disseminated oligodendroglial-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity [corrected]. | journal = J Neurooncol | volume = 124 | issue = 1 | pages = 65-74 | month = Aug | year = 2015 | doi = 10.1007/s11060-015-1735-z | PMID = 25672644 }}</ref>
* 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==
* 1p loss (or 1p/19q loss)<ref>{{Cite journal  | last1 = Bourne | first1 = TD. | last2 = Mandell | first2 = JW. | last3 = Matsumoto | first3 = JA. | last4 = Jane | first4 = JA. | last5 = Lopes | first5 = MB. | title = Primary disseminated leptomeningeal oligodendroglioma with 1p deletion. Case report. | journal = J Neurosurg | volume = 105 | issue = 6 Suppl | pages = 465-9 | month = Dec | year = 2006 | doi = 10.3171/ped.2006.105.6.465 | PMID = 17184079 }}</ref>
** 1p loss is usu. absent in [[pilocytic astrocytoma]]
**Cases without 1p/19q loss reported.<ref>{{Cite journal  | last1 = Preuss | first1 = M. | last2 = Christiansen | first2 = H. | last3 = Merkenschlager | first3 = A. | last4 = Hirsch | first4 = FW. | last5 = Kiess | first5 = W. | last6 = Müller | first6 = W. | last7 = Kästner | first7 = S. | last8 = Henssler | first8 = A. | last9 = Pekrun | first9 = A. | title = Disseminated oligodendroglial-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity [corrected]. | journal = J Neurooncol | volume = 124 | issue = 1 | pages = 65-74 | month = Aug | year = 2015 | doi = 10.1007/s11060-015-1735-z | PMID = 25672644 }}</ref>
*KIAA1549-BRAF fusions.<ref>{{Cite journal  | last1 = Rodriguez | first1 = FJ. | last2 = Schniederjan | first2 = MJ. | last3 = Nicolaides | first3 = T. | last4 = Tihan | first4 = T. | last5 = Burger | first5 = PC. | last6 = Perry | first6 = A. | title = High rate of concurrent BRAF-KIAA1549 gene fusion and 1p deletion in disseminated oligodendroglioma-like leptomeningeal neoplasms (DOLN). | journal = Acta Neuropathol | volume = 129 | issue = 4 | pages = 609-10 | month = Apr | year = 2015 | doi = 10.1007/s00401-015-1400-9 | PMID = 25720745 }}</ref>
** High frequency of co-occurence of BRAF-KIAA1549 fusions and 1p deletion.
** BRAF-KIAA1549 fusions are usu. absent in [[oligodendroglioma]].<ref>{{Cite journal  | last1 = Gierke | first1 = M. | last2 = Sperveslage | first2 = J. | last3 = Schwab | first3 = D. | last4 = Beschorner | first4 = R. | last5 = Ebinger | first5 = M. | last6 = Schuhmann | first6 = MU. | last7 = Schittenhelm | first7 = J. | title = Analysis of IDH1-R132 mutation, BRAF V600 mutation and KIAA1549-BRAF fusion transcript status in central nervous system tumors supports pediatric tumor classification. | journal = J Cancer Res Clin Oncol | volume =  | issue =  | pages =  | month = Jun | year = 2015 | doi = 10.1007/s00432-015-2006-2 | PMID = 26115961 }}</ref>
*BRAF V600E-negative.
 
==Images==
* Radiopedia.org [[http://radiopaedia.org/cases/disseminated-oligodendroglial-like-leptomeningeal-tumour-of-childhood]]
 
<gallery>
File:Neuropathology case DOLN HE I.jpg | DOLN - HE low mag. (WC/jensflorian)
File:Neuropathology case DOLN HE II.jpg | DOLN - HE intermed mag. (WC/jensflorian)
File:Neuropathology case DOLN HE III.jpg | DOLN - HE high mag. (WC/jensflorian)
</gallery>
 
==DDx==
*[[Oligodendroglioma]]<ref>{{Cite journal  | last1 = Bourne | first1 = TD. | last2 = Mandell | first2 = JW. | last3 = Matsumoto | first3 = JA. | last4 = Jane | first4 = JA. | last5 = Lopes | first5 = MB. | title = Primary disseminated leptomeningeal oligodendroglioma with 1p deletion. Case report. | journal = J Neurosurg | volume = 105 | issue = 6 Suppl | pages = 465-9 | month = Dec | year = 2006 | doi = 10.3171/ped.2006.105.6.465 | PMID = 17184079 }}</ref>
 
==See also==
*[[Neuropathology tumours]].
*[[Pilocytic astrocytoma]].
*[[Oligodendroglioma]].
 
==References==
{{Reflist|2}}
 
[[Category:Diagnosis]]
[[Category:Neuropathology tumours]]

Latest revision as of 10:17, 12 November 2020

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