Hemangiopericytomas of the skull base

Abstract

Object: Intracranial hemangiopericytomas are frequently located along the dural sinuses along the skull base and represent rare, aggressive CNS neoplasms that are difficult to distinguish from meningiomas based on both imaging and gross characteristics. The authors of this study describe 3 patients with these lesions and review the pertinent literature. Methods: Two men and 1 woman, whose median age at the time of the initial presentation was 37 years (range 20-53 years), constitute this series. They underwent multimodal treatment consisting of resection, embolization, radiation therapy, and in 1 case chemotherapy. Results: Two of the 3 patients treated were alive after a mean follow-up of 93 months (range 4-217 months). One patient died 217 months after the initial diagnosis. The longest tumor progression-free interval after the initial or secondary resection was 43 months (range 4-84 months). Conclusions: Hemangiopericytomas have been reclassified as mesenchymal nonmeningothelial tumors. They have an inevitable tendency to recur locally and metastasize distally The mainstay of therapy remains an aggressive attempt to achieve gross-total resection at the initial surgery. Postoperative adjuvant radiotherapy should be offered to all patients, regardless of the degree of resection achieved. Diligent long-term follow-up is paramount as local recurrences and distal metastases can develop sometimes years after the initial treatment.