Intracranial hemangiopericytoma--our experience in 30 years: a series of 43 cases and review of the literature

Abstract

Objective: Meningeal hemangiopericytoma (HPC) is a rare, aggressive central nervous system tumor that tends to invade locally and to metastasize, and has a high rate of recurrence.

Methods: This study presents a retrospective review of patients managed for intracranial HPC at Rome University Hospital.

Results: A total of 43 patients with intracranial HPC were treated from 1980 to 2010. Treatment and follow-up information was available for analysis on 36 patients. The median survival for all patients was 83.5 months after date of diagnosis, with 1-year, 5-year, and 10-year survival rates of 100%, 94.4%, and 72.2%, respectively. Eighteen patients (41.86%) had HPC recurrence. The median time until recurrence was 72.24 months, with 1-year, 5-year, and 10-year progression-free survival rates of 98%, 51%, and 29%, respectively. Five patients (11.62%) developed extracranial metastasis. Patients undergoing any form of adjuvant radiation treatment, including external beam radiotherapy, Gamma Knife radiosurgery, and/or proton beam therapy, had no longer median overall survival (OS) (178 vs. 154 months, respectively; P = .2); but did have a significantly improved recurrence-free interval (108 vs. 64 months; P = .04) compared with patients who did not undergo radiation treatment. Tumor characteristics associated with earlier recurrence included size ≥7 cm (log-rank, P < .05) and sinus invasion (log-rank, P < .05).

Conclusions: Strategies combining adjuvant radiation with tumor resection seemed to hinder tumor progression, but had no effect on OS or the development of metastases. Greater extent of resection was associated with increased OS (log-rank, P < .05). Anaplastic HPC was associated with reduced OS and with reduced recurrence interval (log-rank, P < .05).

Keywords: Hemangiopericytoma; Intracranial; Recurrence; Survival; Treatment.