Hemangiopericytomas are rare tumors that behave aggressively with a high rate of local recurrence and distant metastases. With the aim of determining the outcome and response to various treatment modalities, a series of 39 patients who underwent microsurgical resection for primary meningeal hemangiopericytoma over a 24-year period is presented.
Patients with hemangiopericytoma were identified from histopathology records and their medical records were analyzed retrospectively by 2 independent reviewers to collect data on surgical treatment, adjuvant therapy, postoperative course, local or distant recurrence, and follow-up.
Of the 39 patients, 19 were male and 20 were female. Mean patient age was 44.1 years. Thirty-four tumors were intracranial and 5 were spinal. The mean follow-up period was 123 months. Twenty-eight patients developed local recurrence. The recurrence rate at 1, 5, and 15 years was 3.5%, 46%, and 92%, respectively. Extraneural metastasis occurred in 8 patients (26%) at an average of 123 months after initial surgery. Recurrences and metastases were treated by surgical excision, external beam radiation therapy (EBRT), chemotherapy, and/or stereotactic radiosurgery. Adjuvant EBRT following initial surgery was found to extend the disease-free interval from 154 months to 254 months, although it did not prevent the development of metastasis. In those patients with EBRT and complete resection, the mean recurrence-free interval was found to be 126.3 months longer (p = 0.04) and overall survival 126 months longer than without EBRT. Furthermore, adjusting for resection, patients undergoing EBRT had 0.33 times increased risk of recurrence compared with those who did not (p = 0.03). A majority of patients remained able to live independently despite revision surgery for recurrence.
The mean follow-up of this patient series represents the longest follow-up duration published to date and demonstrates extended survival in a significant number of patients with hemangiopericytoma. Gross-total resection followed by adjuvant EBRT provides patients with the highest probability of an increased recurrence-free interval and overall survival. Prolonged survival justifies long-term follow-up and aggressive treatment of initial, recurrent, and metastatic disease.
Abbreviations used in this paper: EBRT = external beam radiation therapy; GKS = Gamma Knife surgery; GTR = gross-total resection; OS = overall survival.
Address correspondence to: Marco Schiariti, M.D., Department of Neurosurgery, Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Via Francesco Sforza, 35 – 20100, Milan, Italy. email: email@example.com.
Please include this information when citing this paper: published online July 30, 2010; DOI: 10.3171/2010.6.JNS091660.
DufourHMétellusPFuentesSMurraccioleXRégisJFigarella-BrangerD: Meningeal hemangiopericytoma: a retrospective study of 21 patients with special review of postoperative external radiotherapy. Neurosurgery48:756–7632001
DufourH, MétellusP, FuentesS, MurraccioleX, RégisJ, Figarella-BrangerD, : Meningeal hemangiopericytoma: a retrospective study of 21 patients with special review of postoperative external radiotherapy. 48:756–763, 2001)| false
EckerRDMarshWRPollockBEKurtkaya-YapicierOMc-ClellandRScheithauerBW: Hemangiopericytoma in the central nervous system: treatment, pathological features, and long-term follow up in 38 patients. J Neurosurg98:1182–11872003
EckerRD, MarshWR, PollockBE, Kurtkaya-YapicierO, Mc-ClellandR, ScheithauerBW, : Hemangiopericytoma in the central nervous system: treatment, pathological features, and long-term follow up in 38 patients. 98:1182–1187, 2003)| false