Management of recurrent intracranial hemangiopericytoma
ABSTRACT 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.
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ABSTRACT: Background. Intracranial haemangiopericytoma (HPC), a rare malignant tumour, should be distinguished from meningioma and solitary fibrous tumour, which have been considered as separate entities since 1993, according to histopathology and clinical characteristics. Methods. A PUBMED search for "Intracranial Haemangiopericytoma" yielded 176 articles, where 26 were of particular interest for this review article. Case report. Our patient, a 27-year-old man with HPC of grade III according to WHO, presents with an acute intracerebral haematoma, which is extremely rare. Results. Surgery (total resection) is the primary treatment. Long-term close clinical and radiological follow-up is crucial due to the high rate of recurrence and tendency for development of metastasis. Discussion. The effects of postoperative radiotherapy need further investigation. Besides neurosurgery, radiotherapy should always be considered in both patients with these highly malignant tumours (WHO grade III) and in patients with partial resection or inoperable cases (WHO grade II).Acta oncologica (Stockholm, Sweden) 08/2012; 52(4). DOI:10.3109/0284186X.2012.716163 · 2.27 Impact Factor
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ABSTRACT: Meningeal hemangiopericytomas (M-HPC) are challenging tumors with a high rate of recurrence despite surgical resection and external beam radiotherapy (EBRT). To better understand the role of single-fraction stereotactic radiosurgery (SRS) for patients with M-HPC, we reviewed our experience with 22 patients (12 men, 10 women) from 1990 until 2010. Twelve patients (55 %) underwent a single SRS procedure, whereas 10 patients (45 %) had more than one SRS procedure (range 2-6). In total, 47 SRS procedures were performed to treat 64 tumors. Fourteen patients (64 %) had undergone prior EBRT (median dose, 56.0 Gy). Follow-up after the initial SRS (median, 66 months) was censored at the time of death (n = 15) or last clinical evaluation (n = 7). Eleven patients (50 %) died of intracranial tumor progression (n = 10) or treatment-related complications (n = 1). One patient (5 %) died of systemic disease progression. Disease-specific survival (DSS) at 1-, 3- and 5-years after SRS was 96, 82, and 61 %, respectively. Prior EBRT (HR 9.0, 95 % CI 1.1-78.1, p < 0.05) and larger initial tumor volume (HR 1.09, 95 % CI 1.02-1.2, p = 0.02) were associated with worse DSS. Local tumor control (LTC) after SRS at 1-, 3-, and 5-years was 89, 68, and 59 %, respectively. Improved LTC was noted in patients who had not undergone prior EBRT (HR 6.3, 95 % CI 2.1-19.5, p = 0.001). One patient (5 %) had symptomatic radiation-relation complications after SRS. Overall, single-fraction SRS was effective in providing LTC for more than half of recurrent or residual M-HPC at 5-years after the procedure. Repeat SRS is often required secondary to either distant or local tumor progression.Journal of Neuro-Oncology 07/2014; 120(1). DOI:10.1007/s11060-014-1521-3 · 3.12 Impact Factor
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ABSTRACT: Primary intracranial haemangiopericytomas (HPC) are rare, highly vascular tumours with a high propensity for local recurrence and distant metastasis. Optimal treatment includes maximal surgical resection followed by adjuvant radiotherapy. In 2007, new histopathological grading criteria were introduced to differentiate between high grade (World Health Organization [WHO] grade III) and low grade (WHO grade II) tumours. Given the rarity of this tumour, there is a paucity of information regarding the prognostic significance of histological grade. We conducted a retrospective review of our 20year experience in treating 27 patients with HPC at our institution. Statistical analysis to compare overall survival, local recurrence rate and metastatic potential between the two grades were conducted using Kaplan-Meier analysis. The estimated median survival for grade II HPC was 216months and for grade III tumours was 142months. On multivariate analysis, grade II tumours were associated with better survival than grade III lesions (hazard ratio=0.16, 95% confidence interval 0.26-0.95; p=0.044). During the study period, 33% of grade III tumours developed local recurrence compared to 21% of grade II tumours. Metastases were found in 36% of grade II patients and 25% of grade III patients. There was no significant statistical difference in local recurrence rate and metastasis between the two grades. Higher histological grading in HPC is associated with worse overall survival. However based on our series higher histological grading is not associated with higher local recurrence or distant metastatic rates.Journal of Clinical Neuroscience 04/2014; 21(8). DOI:10.1016/j.jocn.2013.11.026 · 1.32 Impact Factor