Mutated IDH1 is a favorable prognostic factor for type 2 gliomatosis cerebri

Mi Jung Kwon, Sung Tae Kim, Mi Jeong Kwon, Doo Sik Kong, Dageun Lee, Sanghui Park, So Young Kang, Ji Young Song, Do Hyun Nam, Yukinari Kato, Yoon La Choi, Yeon Lim Suh

Research output: Contribution to journalArticlepeer-review

23 Scopus citations


The prognostic significance of IDH1 mutations has been demonstrated in gliomas. It is unclear whether IDH1 mutation is also a prognostic factor in gliomatosis cerebri (GC). Primary GCs can be grouped into type 1 GCs, which have the classical diffuse growth pattern without mass formation, and type 2 GCs, which form neoplastic masses in addition to classic diffuse lesions. In this study, the prognostic relevance of IDH1/2 mutations in 74 GCs (43 type 1 and 31 type 2) was evaluated. We detected 33 (44.6%) IDH1 mutations, including R132H and R132S, by bidirectional Sanger sequencing. No mutations were detected in IDH2. The percentage of 2-year overall survival for wild-type IDH1 patients was 46 vs. 72% for patients with IDH1-mutated tumors. Mutations of IDH1 were strongly correlated with both increased overall survival (OS) and progression-free survival (PFS) in patients with type 2 GCs, and IDH1 mutations were also an independent prognostic factor predicting increased OS and PFS in type 2 GC patients in multivariate analysis. However, IDH1 mutations did not correlate with survival outcomes in patients with type 1 GCs. Finally, the subgroup of GC, which has IDH1 wild-type and additional solid component showed the worst prognosis.

Original languageEnglish
Pages (from-to)307-317
Number of pages11
JournalBrain Pathology
Issue number3
StatePublished - May 2012


  • Gliomatosis cerebri
  • IDH1
  • IDH2
  • Mutations
  • Prognostic factor


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