Article
Primary gliosarcoma – demographic, clinical, radiographic and molecular-genetic characteristics and differences with primary glioblastoma
Gliosarkome – demographische, klinische, radiologische und molekulargenetische Charakteristika von Gliosarkome und Unterschiede zu Glioblastomen
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Published: | June 26, 2020 |
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Objective: Primary Gliosarcoma (PGS) is a very rare variant of malignant primary brain tumors. Accordingly, data on the epidemiology and natural history of PGS is scarce. The treatment strategies for PGS are based on the studies on primary glioblastoma (PGB) patients. We aimed at analyzing the basic characteristics and clinical course of PGS patients and eventual differences to PGB.
Methods: All consecutive cases with PGS and PGB treated at our university hospital between January 2001 and December 2018, were eligible for this study. Demographic, clinical, radiographic and histological characteristics were collected. Univariate, multivariate (MVA) and propensity score matching analyses were performed.
Results: The final analysis consisted of 56 PGS and 1249 PGB cases. In univariate analysis, there was a trend towards longer OS under temozolomide (TMZ)-therapy, as compared to radiotherapy without TMZ (p=0.0510). But in MVA, only higher patients’ age (p=0.003), preoperative KPS<80% (p=0.035) and larger tumor size (p=0.046), but not TMZ-therapy (p=0.576) were independently associated with the OS of PGS. Compared to PGB, PGS patients showed temporal predilection (p=0.006), higher rates of concomitant eccentric cysts (p=0.001), lower profiles of GFAP staining (p=0.006) and MGMT methylation (p=0.038), as well as higher rates of P53 mutations (p=0.006). In the MVA for OS predictors, PGS patients showed significantly poorer outcome, when compared to the whole PGB cohort (p=0.043). However, when restricting the PGB cohort to the IDH1-WT cases, the outcome difference was no more significant.
Conclusion: Poorer outcome and response to TMZ-therapy in PGS patients is most likely related to less favorable IDH1 and MGMT methylation status of these individuals. As to other differences with PGB, there are PGS-specific anatomic characteristics like temporal predilection and presence of eccentric cysts.