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脊柱的血管外皮细胞瘤:26 例患者的临床特征、分类、治疗和长期随访。

Hemangiopericytomas in the spine: clinical features, classification, treatment, and long-term follow-up in 26 patients.

机构信息

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.

出版信息

Neurosurgery. 2013 Jan;72(1):16-24; discussion 24. doi: 10.1227/NEU.0b013e3182752f50.

Abstract

BACKGROUND

Intraspinal hemangiopericytoma (HPC) is a rare and malignant extra-axial tumor with a strong tendency to recur and metastasize. There is a paucity in the literature of large case series of patients with intraspinal HPCs.

OBJECTIVE

We retrospectively analyzed the clinical radiological and histological features, classification, and treatment of 26 patients with HPCs in the spine.

METHODS

Twenty-six patients with HPCs in the spine were treated at our institution between 1987 and 2010. Medical records were reviewed retrospectively to collect data on the clinical features, tumor morphology, surgical resection, recurrence, and follow-up.

RESULTS

The 26 patients were predominantly male, and the mean age at diagnosis was 33.8 years. The intraspinal HPCs were divided into 3 types and 5 subtypes. Most of them involved the neighboring segments and/or caused bony erosion. All tumors were immunohistochemically positive for vimentin and negative for epithelial membrane antigen. All patients underwent at least 1 surgery, and most of them received postsurgical radiotherapy. The 5-year Kaplan-Meier rate of survival was 76%. The 5-year recurrence-free rate of survival was 29.4%. Only the tumor pathological grade was significantly associated with survival time and recurrence.

CONCLUSION

High-grade tumors had a shorter survival time and recurred earlier than low-grade tumors. Surgical removal and postoperative radiotherapy are critical for the treatment of intraspinal HPCs. However, total resection may not necessary for these tumors. Stereotactic radiosurgery may be a good alternative to control the recurrent lesions.

摘要

背景

椎管内血管外皮细胞瘤(HPC)是一种罕见的恶性外轴肿瘤,具有较强的复发和转移倾向。文献中缺乏大量的椎管内 HPC 患者的病例系列研究。

目的

我们回顾性分析了 26 例椎管内 HPC 患者的临床影像学和组织学特征、分类和治疗。

方法

1987 年至 2010 年,我们机构治疗了 26 例椎管内 HPC 患者。回顾性分析病历资料,收集患者的临床特征、肿瘤形态、手术切除、复发和随访等数据。

结果

26 例患者以男性为主,诊断时的平均年龄为 33.8 岁。椎管内 HPC 分为 3 型和 5 个亚型。大多数累及相邻节段和/或引起骨侵蚀。所有肿瘤免疫组化均为波形蛋白阳性,上皮膜抗原阴性。所有患者均至少接受了 1 次手术,大多数患者术后接受了放疗。5 年 Kaplan-Meier 生存率为 76%。5 年无复发生存率为 29.4%。只有肿瘤病理分级与生存时间和复发显著相关。

结论

高级别肿瘤的生存时间较短,复发较早。手术切除和术后放疗对治疗椎管内 HPC 至关重要。然而,这些肿瘤并不一定需要完全切除。立体定向放射外科可能是控制复发病灶的一种较好的选择。

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