Hemangiopericytoma: long-term outcome revisited

Clinical article

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Object

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.

Methods

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.

Results

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.

Conclusions

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.

Article Information

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: mp_schiariti@hotmail.com.

Please include this information when citing this paper: published online July 30, 2010; DOI: 10.3171/2010.6.JNS091660.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Probability of recurrence (y axis) in relation to the number of months (x axis) after the first operation. Recurrence was dated at the identification of local tumor growth identified on MR imaging. Rates were calculated using the Kaplan-Meier method. The average period before the first recurrence was 80 months (median 61 months).

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    Probability of developing metastases in relation to the number of months after the first operation for the primary tumor. Rates were calculated using the Kaplan-Meier method. Nine patients suffered metastases at an average of 123 months after initial surgery.

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    Probability of survival in relation to the number of months after the first operation. Rates were calculated with the Kaplan-Meier method. Tumor-related deaths were used as end points. The average OS was 216 months.

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    Probability of survival in patients with low- and high-grade tumors in relation to the number of months after the first operation. Rates were calculated with the Kaplan-Meier method. The average survival was 142 and 256 months in the low- and high-grade tumor groups, respectively (a difference of 114 months; p = 0.03).

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    Probability of recurrence in patients with complete and incomplete excision in relation to the number of months after the first operation. Rates were calculated using the Kaplan-Meier method. The average period before the first recurrence was 117 and 54 months in the complete and incomplete resection groups, respectively (p = 0.0045).

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    Probability of survival in patients with and without radiotherapy (EBRT) in relation to the number of months after the first operation. Rates were calculated using the Kaplan-Meier method. The average length of survival was 254 and 154 months in the EBRT and non-EBRT groups, respectively (p = 0.2).

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    Probability of recurrence in patients who underwent EBRT adjusting for surgical excision. Rates were calculated using Cox regression analysis (p = 0.03).

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