Management of recurrent intracranial hemangiopericytoma

Intracranial hemangiopericytoma (HPC) is an aggressive meningothelial neoplasm. A particularly challenging aspect of management of patients with HPC is optimizing treatment for recurrence, progression, and extracranial metastasis. Here we describe a modern cohort of patients with recurrent HPC to better understand treatment strategies that may improve outcome. Patients managed at UCSF for recurrent intracranial HPC were compiled into a single database based on a retrospective review of patient records, including operative, radiologic, and clinic reports. Cox regression was performed to determine factors that independently predicted treatment outcomes. At UCSF, 14 patients with available treatment and follow-up data were seen for management of HPC recurrence. Eight patients underwent repeat surgical resection, of whom four received adjuvant external beam radiotherapy (EBRT), one received additional Gamma Knife radiosurgery (GKS), and one received brachytherapy. Radiosurgical intervention alone was utilized for recurrence in six patients, with four receiving GKS and two receiving CyberKnife. Nine patients suffered a second recurrence at a median time of 3.5 years following reintervention. Nine patients died following reintervention, with a median survival of 7.9 years following intervention for recurrence. In univariate analysis, factors associated with increased time to second recurrence included non-posterior fossa location (log rank, p < 0.05) and surgical resection with adjuvant EBRT (log rank, p < 0.05). The addition of adjuvant EBRT to surgical resection similarly extended overall survival compared to surgical resection alone (log rank, p < 0.05). GKS was associated with earlier second recurrence compared to surgically based strategies (log rank, p < 0.05). We conclude that when combined with surgical resection, EBRT appears promising in the extension of second recurrence-free survival and overall survival. This multimodality approach also appears to outperform GKS in extending time to second recurrence. Accordingly, when safe and feasible, surgical resection of recurrent HPC with adjuvant EBRT should be the first steps in management.