Long term outcomes in malignant hemangiopericytoma

Abstract

9072 Background: Malignant hemangiopericytoma (MH) is a rare entity, arising from the capillary pericyte. Limited data is available regarding its clinical behavior and outcome. We present a series of patients with long term follow up period. Methods: Clinical characteristics, pathologic data and treatment of 14 patients diagnosed with MH between 1972 and 1992, at our centre, were reviewed retrospectively. All cases were biopsy proven. Survival was determined by using Kaplan-Meier curve. Results: The median age was 49 yrs. (range 34 - 74) and male: female ratio was 1:6. The primary disease sites were as follows: intracranial meningeal 5 (35.7%), extremities 3 (21.4%), uterus 3 (21.4%), metastatic 2 (14.3%), sinonasal 1 (7.1%). Thirteen patients had complete surgical resection of tumor at initial presentation. Two patients got post operative RT. No patient got adjuvant chemotherapy. Two patients got chemotherapy on disease recurrence. Median survival was 14 years. Out of 12 patients with localized disease, 3 had recurrence. 2 patients with intracranial meningeal disease at diagnosis recurred 6.7 and 3.3 years after initial surgery while 1 patient with extremity disease at initial diagnosis recurred after 10.5 years. None of the recurred patients had initial post operative RT. All the patients who recurred and those with metastatic disease at diagnosis died. One patient died without recurrence. Recurrence free survival was 6.5 years. Median survival after recurrence was 3.5 years. Fourteen year cause specific survival was 54%. Conclusions: Hemangiopericytoma is a disease of middle age with female preponderance. Surgical resection is still the main treatment with about 54% of patients surviving 14 years in our series (median is not reached). Patients with localized disease can recur several years after initial treatment. In our series median survival after recurrence was 3.5 years, which is longer than reported in other studies. Role of radiation and systemic therapy needs to be defined in this disease. No significant financial relationships to disclose.