To describe the outcome of patients diagnosed with central nervous system haemangiopericytoma (HPC) or solitary fibrous tumour (SFT) and identify factors that may influence recurrence and survival. Between January 2000 and September 2016, a retrospective search identified 55 HPCs/SFTs. The patients underwent a total of 101 surgical resections and 56.9% received radiation therapy. Median follow-up was 7.8 years. 28 patients (50.9%) were re-operated for tumour recurrence. At the end of the study, 21 patients (42%) had no residual tumour on the last scan. Surgical recurrence-free survival at 5 years was 75.2%, 95% CI [63.3-89.3] and, the median surgical recurrence-free survival was 7.4 years. In the adjusted analysis, venous sinus invasion (present vs. absent) (HR 3.39, 95% CI [1.16, 9.93], p = 0.026), completeness of resection (HR 0.38, 95% CI [0.15-0.97], p = 0.042) and tumour subtype (SFT vs. HPC) (HR 3.02, 95% CI[1.02, 8.91], p = 0.045) were established as independent prognostic factors. At the end of the study, 25 patients were deceased (45.5%). and only 15 patients (27.3%) had no residual tumour on the last scan and were alive. Overall survival at 5 years was 80.2, 95% CI [69.3-92.8] and the median overall survival was 13.1 years. None of the investigated variables was associated with overall survival. Patients who received radiation therapy demonstrated neither a reduced risk of surgical recurrence (p = 0.370) nor a longer overall survival (p = 1.000). SFTs/HPCs are associated with a significant risk of recurrence that may reduce the survival of the patients. Total tumour resection upon initial surgery is associated with a lower risk of relapse but not with a prolonged survival. We did not observe a significant improvement in any of the clinical outcomes after radiation therapy.
Keywords: Haemangiopericytoma; Prognostic factors; Radiotherapy; Recurrence; Solitary fibrous tumour.