Meningeal Hemangiopericytomas: Clinical Features, Treatment and Long-term Outcome

Lee, June Ho; Hwang, Sung Kyun; Gwak, Ho Shin; Kim, Dong Gyu; Paek, Sun Ha; Kim, Hyun Jib; Jung, Hee Won

Abstract

OBJECTIVES
Meningeal hemangiopericytoma is a rare tumor. Clinical and radiological features are similar to those of an ordinary meningioma. But its biological behavior is quite different from those of a meningioma as it brings profuse bleeding in the operation field, frequent recurrence and metastasis to other systemic organs even in the case of gross total resection. In order to find out the prognostic factors and to compare long-term outcome after various treatment modalities, the authors reviewed consecutive 20 operated cases of meningeal hemangiopericytoma to characterize their clinical features, surgical outcomes and effectiveness of the radiation therapy.
METHODS
Twenty patients with a hemangiopericytoma were treated between 1982 and 1999 in our department. They are composed of 13 patients of hemangiopericytoma and 7 patients who were initially diagnosed as angioblastic type meningioma and then confirmed as a hemangiopericytoma after review of their previous histopathology slides. The mean follow-up period was 99 months(1-256 months). The long-term outcomes after various treatment modalities were evaluated according to recurrence-free survival and image follow-up. Recurrence-free survival curves are compared between the patient groups according to the extent of removal and radiation therapy.
RESULTS
There were 10 cases of recurrence and 4 cases of distant metastases; lung, liver and femur(2 cases) respectively. The 5-year recurrence rate was 37%, while the 10-year recurrence rose to 77%. There was also statistically significant difference of median recurrence-free survival between the completely-resected group(Simpson grade 1 or 2) and partially-resected group(Simpson grade 3 or 4 or 5); 137 months compared to 47 months, respectively(p=0.009). The median recurrence-free period after subtotal resection of tumor and postoperative radiotherapy was 47 months compared to 117 months of the patients who underwent gross total resection of tumor and did not receive radiotherapy. But radiation therapy alone did not show significant difference in recurrence-free survival. Marked tumor volume reduction and easy removal of tumors without bleeding were found in 2 recurred cases.
CONCLUSION
The extent of tumor resection and presence of metastasis are the most important factors related to long-term outcome of the patients with hemangiopericytoma. Radiation therapy after the first operation did not have a role in extending the recurrence-free survival, but it affected favorably to decrease the size of residual mass and intraoperative bleeding during the second operation.