Patterns of care and outcomes in patients with intracranial hemangiopericytomas: The Memorial Sloan-Kettering Cancer Center (MSKCC) experience
e13011 Background: Intracranial hemangiopericytomas are rare primary brain tumors with a tendency to metastasize. Available literature is restricted to small series of patients, and little is known regarding optimal clinical management and disease course, particularly in the targeted therapy era. Methods: Retrospective review of all patients with intracranial hemangiopericytoma seen at MSKCC from January 1, 1990 to December 31, 2008. Patients were included if histology was reviewed at MSKCC, and if clinical information was deemed sufficient for the analysis. Results: A total of 32 patients met inclusion criteria. The median age was 43 (range 24–68), median KPS was 80 (range 70–100), 17 were women. Disease was metastatic at presentation in only one patient. Initial treatment consisted of surgical resection in all patients (gross total resection: 14 patients, partial resection: 7, equivocal/unknown extent of surgery: 11). Adjuvant radiotherapy following surgery was given to 21 patients. The median progression-free survival was 65 months; median overall survival was 153 months and the 15-year survival was 48%. Treatment for recurrence included re-resection in 19 patients, additional radiotherapy in 17, and chemotherapy in 10. Regimens used included cytotoxic chemotherapy (6 patients) and targeted therapy (sorafenib: 3 patients; sunitinib: 3; imatinib: 2; erlotinib: 1; sirolimus: 1; bevacizumab: 1). Stable disease was the best observed response to these agents. Metastatic sites throughout disease course included lungs in 7 patients, bone in 10, liver in 3 and chest wall in 2. Immunohistochemistry and molecular analyses are ongoing and updated results will be presented. Conclusions: Hemangiopericytomas can be associated with late recurrences, even in patients completely resected and irradiated. Salvage treatment with surgery and radiotherapy seems effective, although the efficacy of chemotherapy remains to be determined. Given the slow growth rates, the meaning of stable disease while on chemotherapy is uncertain. Several patients in this series received agents targeting PDGFR or VGFR pathways, but such strategies need to be investigated further. No significant financial relationships to disclose.