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眼眶孤立性纤维肿瘤:十例长期随访的临床病理研究。

Orbital solitary fibrous tumor: a clinicopathologic study of ten cases with long-term follow-up.

机构信息

Department of Neuropathology, Huashan Hospital, Fudan University, Shanghai 200040, China.

出版信息

Acta Neurochir (Wien). 2012 Feb;154(2):249-55; discussion 255. doi: 10.1007/s00701-011-1254-4. Epub 2011 Dec 28.

DOI:10.1007/s00701-011-1254-4
PMID:22203231
Abstract

BACKGROUND

Solitary fibrous tumor (SFT) is a rare spindle-cell benign neoplasm and located in orbit. The present research represents case reports of ten patients with orbital SFT.

METHOD

Prognosis was assessed by clinic service and telephone interview. Clinical data were retrieved from the medical records. Immunohistochemistry for CD34, CD99, EMA, HMB-45, Bcl-2, GFAP, S-100, MBP, CK, and MIB-1 was performed. Distributions of time to progression and recurrence between different operative methods were estimated using the Kaplan-Meier method and compared using the log-rank test.

FINDINGS

The ten patients included six men and four women (range: 19-75 years). The most common initial symptom was painless proptosis (n = 6). After the ten initial surgeries, with four requiring gross total resection (GRT) and six requiring subtotal resection (STR), an additional nine were required to remove recurrent tumors. Eighteen samples were obtained for pathological examination (the third sample for case 4 was not achieved). Cellular areas with partial hemangiopericytoma pattern were noted in four samples (4/18); scarce mitosis was noted in six samples (6/18). All SFTs (18/18) were positive for CD34, CD99 and vimentin, while all SFTs (18/18) were negative for EMA, CK, MBP, HBM-45 and GFAP. Bcl-2 was positive in 13/18 cases, while S-100 was negative in 14/18 cases. The MIB-1 labeling index varied from 1% to 3%. Follow-up was available for nine patients with a median of 88 months. All four patients who underwent GTR on initial operation did not recur; while the five patients who underwent STR recurred. The log-rank test showed that the incomplete surgical resection was significantly associated with recurrence (p = 0.015).

CONCLUSIONS

Orbital SFT is a rare mesenchymal tumor and painless proptosis is the most common initial symptom. Immunohistochemistry should be used to differentiate SFT from other tumors. Orbital SFT usually has a non-aggressive histological characteristic, and has a good prognosis if GTR is performed. Postoperative regular and long-term follow-up remains mandatory to monitor recurrence.

摘要

背景

孤立性纤维瘤(SFT)是一种罕见的梭形细胞良性肿瘤,位于眼眶内。本研究报告了 10 例眼眶 SFT 患者的病例报告。

方法

通过临床服务和电话访谈评估预后。从病历中检索临床数据。进行 CD34、CD99、EMA、HMB-45、Bcl-2、GFAP、S-100、MBP、CK 和 MIB-1 的免疫组织化学染色。使用 Kaplan-Meier 法估计不同手术方法之间进展和复发的时间分布,并使用对数秩检验进行比较。

结果

10 例患者包括 6 例男性和 4 例女性(年龄范围:19-75 岁)。最常见的首发症状为无痛性眼球突出(n=6)。在 10 例初次手术后,其中 4 例需要完全切除(GTR),6 例需要次全切除(STR),需要另外 9 例切除复发性肿瘤。对 18 例标本进行了病理检查(第 4 例的第 3 例标本未获得)。4 例标本(4/18)有部分血管外皮细胞瘤样细胞区;6 例标本(6/18)有少量有丝分裂。所有 SFT(18/18)均对 CD34、CD99 和波形蛋白呈阳性,而所有 SFT(18/18)对 EMA、CK、MBP、HBM-45 和 GFAP 均呈阴性。Bcl-2 在 13/18 例中阳性,而 S-100 在 14/18 例中阴性。MIB-1 标记指数为 1%-3%。9 例患者获得随访,中位数为 88 个月。初次手术 GTR 的 4 例患者均无复发,STR 的 5 例患者均复发。对数秩检验显示不完全手术切除与复发显著相关(p=0.015)。

结论

眼眶 SFT 是一种罕见的间叶性肿瘤,无痛性眼球突出是最常见的首发症状。免疫组织化学检查应用于将 SFT 与其他肿瘤区分开来。眼眶 SFT 通常具有非侵袭性的组织学特征,如果进行 GTR,则预后良好。术后定期和长期随访仍然是监测复发的必要条件。

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