1. Varied histomorphology and clinical outcomes of FGFR3-TACC3 fusion gliomas.
- Author
-
McDonald MF, Athukuri P, Anand A, Gopakumar S, Jalali A, Patel AJ, Rao G, Goodman JC, Lu HC, and Mandel JJ
- Subjects
- Humans, Mutation, Protein Kinase Inhibitors, Receptor, Fibroblast Growth Factor, Type 3 genetics, Microtubule-Associated Proteins, Glioma genetics, Glioma surgery, Glioblastoma
- Abstract
Targeted therapies for driver gene fusions in cancers have yielded substantial improvements in care. Here, the authors outline a case series of 6 patients with FGFR3-TACC3 fusion in primary brain tumors ranging from polymorphous low-grade neuroepithelial tumor of the young to papillary glioneuronal tumors and glioblastoma (GBM). Previous studies indicated the FGFR3-TACC3 fusion provides survival benefit to GBM patients. Consistent with this, 2 patients with GBM had unexpectedly good outcomes and survived for 5 and 7 years, respectively. In contrast, 2 patients with initially lower graded tumors survived only 3 years and 1 year, respectively. One patient received erdafitinib, a targeted FGFR inhibitor, for 3 months at late disease recurrence and no response was seen. There were varied histomorphological features, including many cases that lacked the characteristic FGFR3-TACC3 pathology. The findings of this cohort suggest that molecular testing is justified, even for glioma cases lacking classic histopathological signatures. Currently, FGFR3-TACC3 fusion gliomas are often classified on the basis of histopathological features. However, further research is needed to examine whether IDH1/2-wild-type tumors with FGFR3-TACC3 fusion should be classified as a subtype on the basis of this molecular fusion. Because patients with IDH1/2-wild-type GBM with FGFR3-TACC3 fusion have improved survival, routine molecular testing for this mutation in patients enrolled in clinical trials and subsequent stratification may be warranted.
- Published
- 2022
- Full Text
- View/download PDF