Circumscribed/non-diffuse histology confers a better prognosis in H3K27M-mutant gliomas
- 644 Downloads
Diffuse midline gliomas harboring a recurrent H3 lysine 27-to-methionine (p.Lys27Met, H3K27M) constitute a recently defined pathologic entity with a particularly poor prognosis. They mainly occur in midline structures, such as the pons and thalamus of children and young adults, and are highly infiltrative . More recently, case reports have described the H3K27M mutation in circumscribed (non-diffuse) gliomas, many of which are low-grade (e.g., pilocytic astrocytoma, ganglioglioma) [1, 3, 4, 5] (additional references are provided in Online Resource 1). Because of the rarity of these tumors, it is unknown whether they carry the poor clinical outcome ascribed to H3K27M-mutant infiltrating gliomas of the midline. Here, we address this gap in our knowledge by performing an integrated meta-analysis on collated clinical and pathologic data from published studies, data repositories, and collaborative efforts.
A systematic search of the literature was performed from 2012 to November, 2017....
The Venneti lab is supported by Grants from NCI K08 CA181475, Sidney Kimmel, St Baldrick’s, Claire McKenna, Chad Tough, Doris Duke, and Sontag Foundations, and the University of Michigan Pediatric Brain Tumor Initiative. We thank Dr. Kathryn McFadden's for original artwork.
Compliance with ethical standards
Conflict of interest
The authors declare no competing interests.
- 2.Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW (2016) The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 131:803–820. https://doi.org/10.1007/s00401-016-1545-1Google Scholar
- 3.Nguyen AT, Colin C, Nanni-Metellus I, Padovani L, Maurage CA, Varlet P, Miquel C, Uro-Coste E, Godfraind C, Lechapt-Zalcman E et al (2015) Evidence for BRAF V600E and H3F3A K27M double mutations in paediatric glial and glioneuronal tumours. Neuropathol Appl Neurobiol 41:403–408. https://doi.org/10.1111/nan.12196CrossRefPubMedGoogle Scholar
- 4.Orillac C, Thomas C, Dastagirzada Y, Hidalgo ET, Golfinos JG, Zagzag D, Wisoff JH, Karajannis MA, Snuderl M (2016) Pilocytic astrocytoma and glioneuronal tumor with histone H3 K27M mutation. Acta Neuropathol Commun 4:84. https://doi.org/10.1186/s40478-016-0361-0CrossRefPubMedPubMedCentralGoogle Scholar
- 5.Pages M, Beccaria K, Boddaert N, Saffroy R, Besnard A, Castel D, Fina F, Barets D, Barret E, Lacroix L et al (2018) Co-occurrence of histone H3 K27M and BRAF V600E mutations in paediatric midline grade I ganglioglioma. Brain Pathol 28:103–111. https://doi.org/10.1111/bpa.12473CrossRefPubMedGoogle Scholar