Meningeal hemangiopericytomas: Review of an institutional series of 21 cases

Objective: Meningeal hemangiopericytomas (HPCs) are rare tumors that mimic meningiomas radiologically but constitute a distinct clinicopathologic entity. Our operative series of 21 patients with HPCs attempts to review the clinical characteristics of this rare entity. Materials and Methods: This is a retrospective analysis of the case records of all the patients with HPC operated on since 2000 with a minimum of 2 years of follow-up. The following prognostic factors were analyzed for statistical significance - age, sex, location, extent of resection, histopathologic grade, and use of radiotherapy. Results: The study group included 21 patients with a mean age of 38.12 years (range, 13 to 67 y) and with no preferential sex distribution (M:F=11:10). A raised intracranial headache was the predominant presenting complaint (13/21; 61.9%). A predominant skull-base location (13/21, 61.9%) was observed in our series. Gross total removal could be achieved in only 13 cases (61.9%). Majority (18/21; 85.71%) were well-differentiated HPCs. At the end of a mean follow-up period of 4.63 years (range, 2 to 11 y), majority of the patients had a good outcome (16/21, 76.1%) and were in the Glasgow outcome score 1. The mean recurrence-free survival after the first surgery was 3.36 years. Recurrence-free survival with gross total removal (3. 95 y) was superior to subtotal removal (STR) alone (2.4 y) and STR with radiotherapy (2.67 y). Conclusions: HPCs are rare tumors that mimic aggressive meningiomas clinically but have a different histogenesis. HPCs are extremely vascular tumors and more commonly occur at skull-base locations, making radical removal a surgical challenge. Radical surgery is the treatment of choice, and the role of adjuvant therapy as a supplement to STR is yet to be established. Long-term follow-up is mandatory as HPCs carry a risk of local recurrence and distant metastases even many years after diagnosis. © 2013 Wolters Kluwer Health, Inc.