Hemangiopericytoma: long-term outcome revisited. Clinical article.
OBJECTIVE
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.
Schiariti
Marco
M
Department of Neurosurgery, National Hospital for Neurology and Neurosurgery, Queen Square, London. mp_schiariti@hotmail.com
Goetz
Pablo
P
El-Maghraby
Hussien
H
Tailor
Jignesh
J
Kitchen
Neil
N
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006393:Hemangiopericytoma / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D006801:Humans; D008297:Male; D008577:Meningeal Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D008866:Microsurgery / Q000379:methods*; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D019635:Neurosurgical Procedures / Q000379:methods*; D011379:Prognosis; D016634:Radiosurgery; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult
D000970:Antineoplastic Agents