Long-term epilepsy associated tumor

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Long-term epilepsy associated tumors, abbreviated LEAT is an umbrella term for rare tumor entities in patients that do not match current WHO diagnostic criteria.

General

  • Closely associated with Ganglioglioma and DNT.
  • Poor interobserver agreement. [1]
  • Some entities can be molecularly defined.[2] - but some not.

Examples

  • Papillary glioneuronal tumor [3]
  • Angiocentric glioma [4]
  • Isomorphic diffuse glioma [5]
  • Multinodular and vacuolating neuronal tumor of the cerebrum [6]
  • Polymorphous low grade tumor of the young [7]


Criteria

See [8]

  • Seizures <18 years.
  • Temporal lobe location.
  • Mostly benign appearance (as in WHO grade I).

See also

References

  1. ↑ "Review: Challenges in the histopathological classification of ganglioglioma and DNT: microscopic agreement studies and a preliminary genotype-phenotype analysis". Neuropathol Appl Neurobiol 45 (2): 95–107. February 2019. doi:10.1111/nan.12522. PMID 30326153.
  2. ↑ "Papillary glioneuronal tumor (PGNT) exhibits a characteristic methylation profile and fusions involving PRKCA". Acta Neuropathol 137 (5): 837–846. May 2019. doi:10.1007/s00401-019-01969-2. PMID 30759284.
  3. ↑ "Papillary glioneuronal tumour: a review of the literature with two illustrative cases". Br J Neurosurg 27 (3): 401–4. June 2013. doi:10.3109/02688697.2012.741735. PMID 23173837.
  4. ↑ "Rare glial tumors". Handb Clin Neurol 134: 399–415. 2016. doi:10.1016/B978-0-12-802997-8.00024-4. PMID 26948368.
  5. ↑ "Isomorphic diffuse glioma is a morphologically and molecularly distinct tumour entity with recurrent gene fusions of MYBL1 or MYB and a benign disease course". Acta Neuropathol 139 (1): 193–209. January 2020. doi:10.1007/s00401-019-02078-w. PMC 7477753. PMID 31563982. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7477753/.
  6. ↑ "Multinodular and vacuolating neuronal tumor of the cerebrum: Two cases and review of the literature". Clin Neurol Neurosurg 197: 106149. October 2020. doi:10.1016/j.clineuro.2020.106149. PMID 32979644.
  7. ↑ "Polymorphous low-grade neuroepithelial tumor of the young (PLNTY): an epileptogenic neoplasm with oligodendroglioma-like components, aberrant CD34 expression, and genetic alterations involving the MAP kinase pathway". Acta Neuropathol 133 (3): 417–429. March 2017. doi:10.1007/s00401-016-1639-9. PMC 5325850. PMID 27812792. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5325850/.
  8. ↑ "Low-grade developmental and epilepsy associated brain tumors: a critical update 2020". Acta Neuropathol Commun 8 (1): 27. March 2020. doi:10.1186/s40478-020-00904-x. PMC 7063704. PMID 32151273. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7063704/.
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