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中枢神经系统孤立性纤维性肿瘤:5 例具有不同临床表现、病理特征和转归模式的病例报告。

Solitary fibrous tumors of the central nervous system: report of five cases with unusual clinicopathological and outcome patterns.

机构信息

Department of Neurosurgery, North Hospital, University Hospital of Saint-Etienne, France.

出版信息

Acta Neurochir (Wien). 2011 Feb;153(2):377-84. doi: 10.1007/s00701-010-0866-4. Epub 2010 Nov 10.

DOI:10.1007/s00701-010-0866-4
PMID:21063734
Abstract

BACKGROUND

This is a retrospective study of 11 patients harboring a solitary fibrous tumor (SFT) of the central nervous system (CNS), with special emphasis on unusual clinicopathological and outcomes patterns.

METHOD

Between 2000 and 2008, 11 patients harboring CNS SFTs were treated at our institution. Patient charts were retrospectively reviewed and tumor location, clinical presentation, imaging characteristics, extent of resection, dural origin, pathological features, adjuvant treatment, and follow-up data were collected, focusing on five atypical cases (four intracranial and one within the spine).

FINDINGS

One intracranial SFT arose from the sella turcica and relapsed threefold during the 6 years following partial removal. Disease progressed as successive isolated local recurrences treated by subsequent surgical interventions and gamma-knife radiosurgery. The MiB-1 labeling index analysis showed a steady increase in these sequential recurrences (ranging from less than 3% up to 6%) without obvious malignant transformation. The second SFT occurred in the cerebellopontine angle and exhibited a high MiB-1 index (10%) without noticeable features of malignancy. It relapsed twice during the 5 years following gross total resection without demonstrating a more aggressive histological pattern. The third SFT arose from the cerebellar tentorium, widely invaded the lateral sinus and adjacent bone, had a low MiB-1 index, and has not recurred within the 2 years after incomplete resection. The two remaining SFTs presented with unusual clinicoradiological features. We described a extremely rare case of intraventricular SFT, and a case of extradural SFT of the thoracic spine (T8-T9) radiologically consistent with a schwannoma. Immunohistochemistry confirmed that all tumors were SFTs.

CONCLUSIONS

These atypical presentations gave us the opportunity to provide further information about the variability of the clinicoradiological patterns and natural histological course of CNS SFTs.

摘要

背景

这是一项对 11 例中枢神经系统(CNS)孤立性纤维瘤(SFT)患者的回顾性研究,特别强调了不常见的临床病理和结果模式。

方法

在 2000 年至 2008 年期间,我们机构治疗了 11 例 CNS SFT 患者。回顾性分析患者病历,收集肿瘤位置、临床表现、影像学特征、切除范围、硬脑膜起源、病理特征、辅助治疗和随访资料,重点关注 5 例非典型病例(4 例颅内和 1 例脊柱内)。

结果

1 例颅内 SFT 起源于鞍区,在部分切除后 6 年内复发 3 次。疾病进展为连续孤立的局部复发,通过随后的手术干预和伽玛刀放射外科治疗。MiB-1 标记指数分析显示这些连续复发的指数稳步增加(从小于 3%到 6%),没有明显的恶性转化。第 2 例 SFT 发生在桥小脑角,MiB-1 指数较高(10%),但无明显恶性特征。在全切除后 5 年内复发 2 次,组织学表现无明显侵袭性。第 3 例 SFT 起源于小脑幕,广泛侵犯外侧窦和邻近骨骼,MiB-1 指数低,不完全切除后 2 年内未复发。其余 2 例 SFT 表现出不同寻常的临床影像学特征。我们描述了 1 例极为罕见的脑室 SFT 和 1 例胸段脊柱(T8-T9)硬膜外 SFT 病例,影像学上与神经鞘瘤一致。免疫组化证实所有肿瘤均为 SFT。

结论

这些不典型表现为我们提供了进一步了解 CNS SFT 临床病理表现和自然病史变异性的机会。

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