Department of Neurosurgery, National Hospital for Neurology and Neurosurgery, Queen Square, London.
J Neurosurg. 2011 Mar;114(3):747-55. doi: 10.3171/2010.6.JNS091660. Epub 2010 Jul 30.
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.
血管外皮细胞瘤是一种罕见的侵袭性肿瘤，具有较高的局部复发率和远处转移率。为了确定各种治疗方法的结果和反应，我们对 24 年来接受显微手术切除原发性脑膜血管外皮细胞瘤的 39 例患者进行了系列研究。
通过组织病理学记录确定血管外皮细胞瘤患者，并由 2 名独立评审员回顾性分析其病历，以收集手术治疗、辅助治疗、术后过程、局部或远处复发以及随访等数据。
39 例患者中，男性 19 例，女性 20 例。平均患者年龄为 44.1 岁。34 例肿瘤位于颅内，5 例位于脊髓。平均随访时间为 123 个月。28 例患者发生局部复发。1、5 和 15 年的复发率分别为 3.5%、46%和 92%。8 例（26%）患者在初次手术后平均 123 个月出现颅外转移。复发和转移采用手术切除、外照射放疗（EBRT）、化疗和/或立体定向放射外科治疗。研究发现，初次手术后辅助 EBRT 将无疾病间隔从 154 个月延长至 254 个月，尽管它不能预防转移的发生。在接受 EBRT 和完全切除的患者中，无复发生存期平均延长 126.3 个月（p=0.04），总生存期延长 126 个月。此外，在调整了切除程度后，接受 EBRT 的患者复发风险比未接受 EBRT 的患者增加了 0.33 倍（p=0.03）。尽管需要进行复发性手术，但大多数患者仍能独立生活。
本患者系列的平均随访时间代表了迄今为止发表的最长随访时间，并证明了大多数血管外皮细胞瘤患者的生存时间延长。大体全切除后辅助 EBRT 为患者提供了增加无复发生存期和总生存期的最大可能性。长期生存证明了长期随访和积极治疗初始、复发性和转移性疾病的合理性。
