Abstract
BACKGROUND: Anaplastic Hemangiopericytoma (AHPC), now known as Solitary Fibrous Tumor (SFT) Grade III, is a rare biologically aggressive neoplasm with a high rate of recurrence. Owing to its rarity, the existing literature is limited regarding its clinical characteristics, prognostic factors, management, and treatment strategies. In this study, we evaluate the association between patient demographics, clinical variables, and treatment modalities with overall survival in patients with intracranial AHPC/SFT Grade III. METHODS: The National Cancer Database (NCDB) was queried for the clinical and care parameters of patients ≥ 18-years-old diagnosed with AHPC between 2004 and 2017. Multivariable Cox proportional hazards model was implemented to determine factors associated with overall survival. RESULTS: 427 patients were identified with a mean age of 52.6 ± 0.7 years. Most patients were between 40-70 years old (67.0%) with patients ≤ 40-years-old making up 21.3% and those ≥ 70-years-old making up only 11.7%. Overall median survival was 10.8 years. When stratified based on age, those ≤ 40-years-old had increased mean survival compared to those ≥ 70-years-old (10.8 vs. 6.6 years; p < 0.001). On multivariable analysis, increasing age (p = 0.006) and the receipt of chemotherapy was associated with decreased overall survival. In contrast, private insurance and managed care (p = 0.021), treatment with surgery alone (p = 0.003), or combined surgery and radiation therapy (p = 0.001) were independently associated with significantly improved overall survival. CONCLUSION: In this NCDB cohort of intracranial grade III SFT/HPC, improved overall survival was associated with younger age, private insurance status, and treatment with surgery alone or surgery combined with radiation therapy. Chemotherapy was not associated with a survival benefit. Interpretation of treatment effects should be cautious, given the potential for selection bias in chemotherapy utilization and the risk of immortal time bias analyses of radiotherapy. Continued advances in therapeutic strategies are needed to further improve survival in this rare and clinically devastating disease.