Methylation profiling of choroid plexus tumors reveals 3 clinically distinct subgroups

Christian Thomas, Martin Sill, Vincent Ruland, Anika Witten, Stefan Hartung, Uwe Kordes, Astrid Jeibmann, Rudi Beschorner, Kathy Keyvani, Markus Bergmann, Michel Mittelbronn, Torsten Pietsch, Jörg Felsberg, Camelia M. Monoranu, Pascale Varlet, Peter Hauser, Adriana Olar, Richard G. Grundy, Johannes E. Wolff, Andrey KorshunovDavid T. Jones, Melanie Bewerunge-Hudler, Volker Hovestadt, Andreas Von Deimling, Stefan M. Pfister, Werner Paulus, David Capper, Martin Hasselblatt*

*Corresponding author for this work

Research output: Contribution to journalArticleResearchpeer-review

54 Citations (Scopus)


Background Choroid plexus tumors are intraventricular neoplasms derived from the choroid plexus epithelium. A better knowledge of molecular factors involved in choroid plexus tumor biology may aid in identifying patients at risk for recurrence. Methods Methylation profiles were examined in 29 choroid plexus papillomas (CPPs, WHO grade I), 32 atypical choroid plexus papillomas (aCPPs, WHO grade II), and 31 choroid plexus carcinomas (CPCs, WHO grade III) by Illumina Infinium HumanMethylation450 Bead Chip Array. Results Unsupervised hierarchical clustering identified 3 subgroups: methylation cluster 1 (pediatric CPP and aCPP of mainly supratentorial location), methylation cluster 2 (adult CPP and aCPP of mainly infratentorial location), and methylation cluster 3 (pediatric CPP, aCPP, and CPC of supratentorial location). In methylation cluster 3, progression-free survival (PFS) accounted for a mean of 72 months (CI, 55-89 mo), whereas only 1 of 42 tumors of methylation clusters 1 and 2 progressed (P<. 001). On stratification of outcome data according to WHO grade, all CPCs clustered within cluster 3 and were associated with shorter overall survival (mean, 105 mo [CI, 81-128 mo]) and PFS (mean, 55 mo [CI, 36-73 mo]). The aCPP of methylation cluster 3 also progressed frequently (mean, 69 mo [CI, 44-93 mo]), whereas no tumor progression was observed in aCPP of methylation clusters 1 and 2 (P<. 05). Only 1 of 29 CPPs recurred. Conclusions Methylation profiling of choroid plexus tumors reveals 3 distinct subgroups (ie, pediatric low-risk choroid plexus tumors [cluster 1], adult low-risk choroid plexus tumors [cluster 2], and pediatric high-risk choroid plexus tumors [cluster 3]) and may provide useful prognostic information in addition to histopathology.

Original languageEnglish
Pages (from-to)790-796
Number of pages7
Issue number6
Publication statusPublished - 10 Jun 2016
Externally publishedYes


  • atypical choroid plexus papilloma
  • choroid plexus carcinoma
  • copy-number alterations
  • epigenetics
  • prognosis


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