National-level overall survival patterns for molecularly-defined diffuse glioma types in the United States

Abstract

Background Molecularly-defined diffuse glioma types – including IDH-wildtype glioblastoma, IDH-mutant astrocytoma, IDH-mutant 1p/19q-codeleted oligodendroglioma, and H3 K27M-mutant diffuse midline glioma – were incorporated into U.S. cancer registry reporting for individuals with brain tumors beginning in 2018. We leveraged these new data to estimate the national-level overall survival (OS) patterns associated with glioma integrated diagnoses. Methods Individuals diagnosed with diffuse gliomas in 2018 and had brain molecular marker data were identified within the U.S. National Cancer Database. OS was estimated using Kaplan Meier methods and stratified by WHO CNS grade, age, sex, tumor size, treatment, extent of resection, and MGMT promoter methylation. Additionally, the effects of WHO CNS grade were examined among individuals with IDH-wildtype astrocytic gliomas. Results 8,651 individuals were identified. One-year OS was 53.7% for WHO grade 4 IDH-wildtype glioblastomas; 98.0%, 92.4%, and 76.3% for WHO grade 2, 3, and 4 IDH-mutant astrocytomas, respectively; 97.9% and 94.4% for WHO grade 2 and 3 IDH-mutant 1p/19q-codeleted oligodendrogliomas, respectively; and 55.9% for H3 K27M-mutant diffuse midline gliomas. Among IDH-wildtype glioblastomas, median OS was 17.1 months and 12.4 months for methylated and unmethylated MGMT promoters. Additionally, IDH-wildtype diffuse astrocytic gliomas reported as WHO grade 2 or 3 demonstrated longer OS compared to grade 4 tumors (both p<0.001). Conclusions Our findings provide the initial national OS estimates for molecularly-defined diffuse gliomas in the U.S. and illustrate the importance of incorporating such data into cancer registry reporting.

Clinical management and survival outcomes of patients with different molecular subtypes of diffuse gliomas in China (2011–2017): a multicenter retrospective study from CGGA

“…However, the changes in the survival of patients with GBM remain uncertain, because the 3-year survival rate increased from 15% to 17%, whereas the 5-year survival rate decreased from 9% to 6%. The survival of our patients was not inferior to that in other non-Chinese cohorts from developed countries, such as the United Kingdom, Japan, and the United States; moreover, patients with GBM survived even longer [36][37][38][39] .…”

“…For patients with recurrent tumors, 37 LGG-IDHm-1p/19qs, 65 LGG-IDHms, 38 LGG-IDHwts, 141 GBM-IDHwts, and 122 GBM-IDHms were identified. Compared with patients with primary tumors, patients with recurrent DGs had a markedly greater proportion of GBM-IDHm (P < 0.0001, Table 1), thus implying the malignant progression of LGG-IDHm to GBM-IDHm.…”

Clinical updates on gliomas and implications of the 5th edition of the WHO classification of central nervous system tumors

“…Regarding clinical characteristics, the elderly tended to suffer aggressive tumor types, as expected ( 1 ). Epilepsy is most prevalent in glioneuronal tumor ( 14 ) and also common in IDH-mutant glioma ( 19 ). Notably, neurological deficits had a bipolar distribution, which was common in the most aggressive glioblastoma and least aggressive circumscribed glioma.…”

“…However, the majority of these studies have only focused on some subtypes, primarily the most malignant form, glioblastoma, IDH-wildtype. The analysis based on national data of gliomas from the USA showed that glioblastoma, IDH-wildtype had a 1-year survival rate of 53.7%, and astrocytoma, IDH-mutant, WHO grade 4 of 73.6% ( 14 ). Another multicenter study reported that histological glioblastoma and molecular glioblastoma had 26 months and 21 months of OS, respectively ( 18 ).…”

“…However, our understanding of the categorization of gliomas and their diagnostic, therapeutic, and prognostic characteristics is limited since it was all from the research based on the previous classification scheme ( 12 , 13 ). Recently, several studies have attempted to explore and update the characteristics of gliomas according to the WHO CNS5 classification, with conflicting results ( 14 – 18 ).…”