Diffuse gliomas with FGFR3-TACC3 fusion have characteristic histopathological and molecular features - Sorbonne Université Access content directly
Journal Articles Brain Pathology Year : 2018

Diffuse gliomas with FGFR3-TACC3 fusion have characteristic histopathological and molecular features

Alberto Picca
Chiara Villa
  • Function : Author
Anna Lasorella
  • Function : Author
Antonio Iavarone
  • Function : Author

Abstract

Adult glioblastomas, IDH-wildtype represent a heterogeneous group of diseases. They are resistant to conventional treatment by concomitant radiochemotherapy and carry a dismal prognosis. The discovery of oncogenic gene fusions in these tumors has led to prospective targeted treatments, but identification of these rare alterations in practice is challenging. Here, we report a series of 30 adult diffuse gliomas with an in frame FGFR3-TACC3 oncogenic fusion (n = 27 WHO grade IV and n = 3 WHO grade II) as well as their histological and molecular features. We observed recurrent morphological features (monomorphous ovoid nuclei, nuclear palisading and thin parallel cytoplasmic processes, endocrinoid network of thin capillaries) associated with frequent microcalcifications and desmoplasia. We report a constant immunoreactivity for FGFR3, which is a valuable method for screening for the FGFR3-TACC3 fusion with 100% sensitivity and 92% specificity. We confirmed the associated molecular features (typical genetic alterations of glioblastoma, except the absence of EGFR amplification, and an increased frequency of CDK4 and MDM2 amplifications). FGFR3 immunopositivity is a valuable tool to identify gliomas that are likely to harbor the FGFR3-TACC3 fusion for inclusion in targeted therapeutic trials.
Fichier principal
Vignette du fichier
2017_BrainPath_FGFR3_sans marque.pdf (1.33 Mo) Télécharger le fichier
Origin Files produced by the author(s)
Loading...

Dates and versions

hal-01676427 , version 1 (05-01-2018)

Identifiers

Cite

Franck Bielle, Anna-Luisa Di Stefano, David Meyronnet, Alberto Picca, Chiara Villa, et al.. Diffuse gliomas with FGFR3-TACC3 fusion have characteristic histopathological and molecular features. Brain Pathology, In press, ⟨10.1111/bpa.12563⟩. ⟨hal-01676427⟩
631 View
561 Download

Altmetric

Share

Gmail Mastodon Facebook X LinkedIn More