Meningeal hemangiopericytomas: Long-term outcome and biological behavior

BACKGROUND: The authors present a retrospective clinical analysis of meningeal hemangiopericytomas. The long-term outcome and biologic behavior, including local recurrence and extraneural distant metastasis, are elucidated. METHOD: Clinical records and radiologic findings of 31 cases with meningeal hemangiopericytoma treated at Seoul National University Hospital and Asian Medical Center between 1982 and 1999 were carefully reviewed. The final outcome was determined by direct or phone contact and questionnaire by mail. The duration of follow-up was from 1 to 216 months (mean 77). All patients underwent craniotomy and Simpson Grade I or II resection was possible in 24 patients. Conventional radiotherapy was given in 11 patients (in 5 after total excision and in 6 for residual mass) and stereotactic radiosurgery was conducted in 6 cases. RESULTS: Intracranial recurrence was seen in 12 cases (38.7%) and the mean period before the first recurrence was 104 months. The recurrence was at the primary site in 11 cases and diffuse leptomeningeal seeding occurred in the remaining case. The 5-year recurrence free rate was 59.2% and the extent of excision was the significant factor (72.7% in total excision group and 20.8% in the incomplete excision group, p = 0.006). In four patients (12.9%), extraneural metastases developed at an average of 107 months. Six patients died during the follow-up period; however, 2 of these died of unrelated causes. CONCLUSIONS: Complete excision is the most important factor in reducing recurrence. However, recurrence may occur even after complete excision. Careful long-term follow-up is mandatory because of the long disease-free interval.