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颅内脑膜血管外皮细胞瘤:一家三级医疗中心的 10 年经验。

Intracranial meningeal hemangiopericytoma: 10 years experience of a tertiary care Institute.

机构信息

Department of Radiotherapy and Oncology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

出版信息

Acta Neurochir (Wien). 2012 Sep;154(9):1647-51. doi: 10.1007/s00701-012-1442-x. Epub 2012 Jul 12.

Abstract

BACKGROUND

Intracranial meningeal hemangiopericytoma (HPC) represents a rare and aggressive intracranial neoplasm located along the dural sinuses. It constitutes less than 1 % of all intracranial tumors and approximately 2-4 % of all meningeal tumors. The authors present our institute's experience in combined modality management of 15 successive patients of HPC.

METHODS

We retrospectively reviewed 15 patients of HPC treated in our institute from 2001 to 2011. Clinical characteristics and treatment modality, in the form of surgery and radiotherapy, were noted. Statistical analysis was done with regards to recurrence free survival (RFS) and overall survival (OS) using Kaplan-Meier survival analysis.

RESULTS

The median age of the patients was 40.0 years. Nine patients were males and six patients were females. The median duration of symptoms was six months. Headache was the most common presenting symptom followed by vomiting, motor weakness and seizures. Twelve patients underwent total excision while three had subtotal excision. Seven patients had WHO grade II histology tumors and eight patients had grade III histology. Thirteen patients received adjuvant radiotherapy (RT). Median RT dose delivered was 50 Gy. Five patients developed local recurrence. One patient had distant metastases. Median duration of RFS was 68 months.

CONCLUSIONS

HPCs are aggressive tumors. The mainstay of therapy is gross total resection at the initial surgery. Postoperative adjuvant RT should be offered to all patients, regardless of the degree of resection achieved. Long-term follow-up is important as local recurrences and distant metastases can develop years after the initial treatment.

摘要

背景

颅内脑膜血管外皮细胞瘤(HPC)是一种罕见且侵袭性的颅内肿瘤,位于硬脑膜窦沿线。它构成了所有颅内肿瘤的不到 1%,约占脑膜瘤的 2-4%。作者介绍了他们所在机构对 15 例连续 HPC 患者进行联合治疗模式的经验。

方法

作者回顾性分析了 2001 年至 2011 年在他们所在机构治疗的 15 例 HPC 患者。记录了临床特征和治疗方式,包括手术和放疗。采用 Kaplan-Meier 生存分析对无复发生存(RFS)和总生存(OS)进行统计学分析。

结果

患者的中位年龄为 40.0 岁。9 例为男性,6 例为女性。中位症状持续时间为 6 个月。头痛是最常见的首发症状,其次是呕吐、运动无力和癫痫发作。12 例患者行全切除,3 例患者行次全切除。7 例患者组织学分级为 II 级,8 例患者组织学分级为 III 级。13 例患者接受辅助放疗(RT)。中位放疗剂量为 50Gy。5 例患者出现局部复发。1 例患者发生远处转移。RFS 的中位时间为 68 个月。

结论

HPC 是侵袭性肿瘤。初始手术的主要治疗方法是大体全切除。无论切除程度如何,所有患者术后均应接受辅助放疗。长期随访很重要,因为局部复发和远处转移可能在初始治疗后数年发生。

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