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Objective: Skull base chondrosarcoma is a rare tumour usually treated by surgery and proton therapy. However, as mortality rate is very low and treatment complications are frequent, a less aggressive therapeutic strategy could be considered. The objective of this study was to compare the results of surgery only vs surgery and adjuvant proton therapy, in terms of survival and treatment adverse effects, based on a retrospective series. Methods: Monocentric retrospective study at a tertiary care centre. All patients treated for a skull base grade I and II chondrosarcoma were included. We collected data concerning surgical and proton therapy treatment and up-to-date follow-up, including Common Terminology Criteria for Adverse Events (CTCAE) scores. Results: 47 patients (23M/24F) were operated on between 2002 and 2015; mean age at diagnosis was 47 years-old (10–85). Petroclival and anterior skull base locations were found in 34 and 13 patients, respectively. Gross total resection was achieved in 17 cases (36%) and partial in 30 (64%). Adjuvant proton therapy (mean total dose 70 GyRBE,1.8 GyRBE/day) was administered in 23 cases. Overall mean follow-up was 91 months (7–182). Of the patients treated by surgery only, 8 (34%) experienced residual tumour progression (mean delay 51 months) and 5 received second-line proton therapy. Adjuvant proton therapy was associated with a significantly lower rate of relapse (11%; p = 0.01). There was no significant difference in 10-year disease specific survival between patients initially treated with or without adjuvant proton therapy (100% vs 89.8%, p = 0.14). Difference in high-grade toxicity was not statistically significant between patients in both groups (25% (7) vs 11% (5), p = 0.10). The most frequent adverse effect of proton therapy was sensorineural hearing loss (39%). Conclusion: Long-term disease specific survival was not significantly lower in patients without adjuvant proton therapy, but they experienced less adverse effects. We believe a surgery only strategy could be discussed, delaying as much as possible proton therapy in cases of relapse. Further prospective studies are needed to validate this more conservative strategy in skull base chondrosarcoma. [ABSTRACT FROM AUTHOR]

Background: A very small proportion of patients diagnosed with glioblastoma (GBM) survive more than 3 years. Isocitrate dehydrogenase 1 or 2 (IDH1/2) mutations define a small subgroup of GBM patients with favourable prognosis. However, it remains controversial whether long-term survivors (LTS) are found among those IDH1/2 mutated patients. Methods: We retrospectively analyzed 207 GBM patients followed at Lariboisière Hospital (Paris) between 2005 and 2010. Clinical parameters were obtained from medical records. Mutations of IDH1/2 were analyzed in these patients, by immunohistochemistry for the R132H mutation of IDH1 and by high-resolution melting-curve analysis, followed by Sanger sequencing for IDH1 and IDH2 exon 4 mutations. Mutation rates in LTS and non-LTS groups were compared by Chi square Pearson test. Results: Seventeen patients with survival >3 years were identified (8.2% of the total series). The median overall survival in long-term survivors was 4.6 years. Subgroup analysis found that the median age at diagnosis was significantly higher for non long-term survivors (non-LTS) compared to LTS (60 versus 51 years, p <0.03). The difference in the rate of IDH mutation between non-LTS and LTS was statistically not significant (1.16% versus 5.9%, p = 0.144). Among LTS, 10 out of 16 tumors presented a methylation of MGMT promoter. Conclusions: This study confirms that long-term survival in GBM patients is if at all only weakly correlated to IDH-mutation. [ABSTRACT FROM AUTHOR]