Gliomatosis cerebri classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

Gliomatosis cerebri may be classified into several subtypes based on the origin (primary and secondary) and the type of tumor cell (astrocytic, oligodendroglial, and mixed).

Classification

Based on the origin

Gliomatosis cerebri may be classified based on the origin into two subtypes:[1][2][3][4]

Type of gliomatosis cerebri Characteristic features

Type 1 (Primary)

  • No pre-existing primary cerebral neoplastic lesion (de novo)
  • No discrete mass
  • Diffuse spread of tumor
  • More common
  • Median age at diagnosis is 56 years
  • Mean survival time was longer than type 2 (24 months)
  • Predominantly low-grade

Type 2 (Secondary)

  • Results from a pre-existing cerebral glioma
  • Discrete mass with diffuse CNS involvement
  • Less common
  • Median age at diagnosis is 44 years
  • Mean survival time was shorter than type 1 (21 months)
  • Predominantly high-grade
  • IDH1 mutation is more common

Based on the type of tumor cell

Gliomatosis cerebri may be classified based on the predominant tumor cell type into three sub-types:[4]

  • Astrocytic - it is considered a distinct entity of astrocytic glioma as per the latest WHO classification for CNS tumors.[5]
  • Oligodendroglial - very few cases of isolated oligodendroglial GC has been reported. No difference in the signs and symptoms are noted.[6]
  • Mixed - more common than isolated oligodendroglial GC.

References

  1. Classification of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri
  2. Brandão RA, de Carvalho GT, de Azeredo Coutinho CA, Christo PP, Santiago CF, Santos Mdo C; et al. (2011). "Gliomatosis cerebri: diagnostic considerations in three cases". Neurol India. 59 (1): 122–5. doi:10.4103/0028-3886.76892. PMID 21339680.
  3. Park S, Suh YL, Nam DH, Kim ST (2009). "Gliomatosis cerebri: clinicopathologic study of 33 cases and comparison of mass forming and diffuse types". Clin Neuropathol. 28 (2): 73–82. PMID 19353837.
  4. 4.0 4.1 Sanson M, Cartalat-Carel S, Taillibert S, Napolitano M, Djafari L, Cougnard J; et al. (2004). "Initial chemotherapy in gliomatosis cerebri". Neurology. 63 (2): 270–5. PMID 15277619.
  5. "Gliomatosis cerebri: no evidence for a separate brain tumor entity".
  6. "Oligodendroglial gliomatosis cerebri".


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