Clinical features and surgical outcomes of primary intracranial synovial sarcomas

Objective

Primary intracranial synovial sarcomas (PISSs) are extremely rare, with only 30 reported cases in the literature. The aim of this study is to outline the clinical characteristics of PISSs, examine prognostic risk factors, and attempt to develop an optimal treatment strategy based on the available data.

Methods

We included a total of 33 PISS cases, comprising 3 patients from our institution and 30 patients documented in the literature. We collated clinical data from these cases and conducted an analysis of prognostic risk factors using Cox proportional hazards models and Kaplan-Meier methods.

Results

The cohort consisted of 21 males and 12 females, with an average age of 30.0 ± 18.5 years (ranging from 1 to 81 years). Among these cases, gross total resection (GTR) was performed in 21 (63.6 %) cases, non-GTR in 11 (33.3 %), and biopsy in 1 (3.0 %) case. Additionally, 25 patients (78.1 %) received RT (Radiotherapy), and 6 patients (18.8 %) underwent CT (Chemotherapy). Over a mean follow-up period of 11.5 ± 14.1 months (ranging from 2 to 84 months), tumor recurrence was observed in 27 patients (90 %), and 21 patients (70 %) died, with an average follow-up duration of 18.9 ± 21.6 months (ranging from 2 to 105 months). Multivariate Cox regression analysis revealed that non-GTR and no RT was independent adverse factors for both PFS (Progression-Free Survival) and OS (Overall Survival). Kaplan-Meier analysis revealed that non-GTR (p = 0.0001) and the absence of radiotherapy (p < 0.0001) were predictive of shorter OS.

Conclusion

In our study, we observed that PISSs predominantly affect young males, and the prognosis of this disease is relatively poor. Our statistical analysis revealed that GTR and postoperative radiotherapy are beneficial for both PFS and OS. Therefore, we recommend GTR combined with postoperative radiotherapy as the standard treatment approach.