Malignant hemangiopericytoma: A study of long-term outcomes

Abstract

9560 Background: Malignant hemangiopericytoma (MH) is a rare disease with limited data on long term clinical outcomes. We presented a poster at ASCO 2005 annual meeting, outlining the long term follow up of 14 patients. We reviewed the data from 10 additional patients from our affiliated center and present here a combined analysis. Methods: Clinical characteristics, pathologic and treatment data of 24 patients diagnosed with MH between 1972 and 1995 was reviewed. All cases were histologically proven. Survival was determined by using Kaplan-Meier curve. Results: The median age was 52 yrs. (range 28 - 74). Male: female ratio was 1: 2.4. Patients presented with involvement of following primary sites: cranial/meningeal 8 (33.34%), extremities 4 (16.7%), uterus 3 (12.5%), metastatic 2 (8.3%), retroperitoneal 2 (8.3%), pelvis 2 (8.3%), sinonasal, middle ear and thyroid 1 each (4.2% each). 20 patients had definitive surgical resection at initial presentation. 4 patients received post operative radiation therapy (RT). No patient received adjuvant chemotherapy. 7 out of 8 patients who recurred died of disease. One patient who was alive 5 years after recurrence got stereotactic RT to localized recurrence. At disease recurrence, 2 patients received chemotherapy and RT, while 5 were treated with surgery and/or RT. 4 out of 8 patients with cranial/meningeal disease at diagnosis recurred. Two of them recurred after 6 years. Only one of the 4 recurred patients had post operative adjuvant RT. Median recurrence free survival was 5.25 years. Median survival after recurrence was 3.28 years. Fourteen year cause specific survival was 55%. Conclusions: Combined analysis of 24 patients shows that complete surgical resection is the main treatment with 55% of patients living at 14 years (median is not reached). Compared to our prior report, female preponderance was less marked. Radiation may play some part in adjuvant setting, but its role is not clearly defined. Role of systemic treatment is not clear. In our series 40% recurred after definitive treatment. Recurrences can occur several years after the initial diagnosis and definitive treatment. There is a need to develop effective adjuvant systemic treatments. No significant financial relationships to disclose.