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脑膜(myo)纤维母细胞瘤性肿瘤与孤立性纤维肿瘤相关,并伴有恶性神经母细胞瘤成分。

A meningeal myofibroblastic neoplasm related to solitary fibrous tumour and associated with a malignant neuroblastic element.

机构信息

Department of Histopathology, Derriford Hospital, Plymouth, Devon, UK.

出版信息

J Clin Pathol. 2010 Feb;63(2):180-3. doi: 10.1136/jcp.2009.067678.

DOI:10.1136/jcp.2009.067678
PMID:20154042
Abstract

BACKGROUND

Solitary fibrous tumour (SFT) is a rare mesenchymal tumour now described at many locations, including the meninges. Intracranial SFT closely resembles meningioma clinically and radiologically, and, like meningioma, reports of meningeal SFT suggest a relatively benign behaviour after complete resection. Histopathological features distinguishing SFT from meningioma include variable cellularity, spindle cells arranged in fascicles, staghorn blood vessels and immunopositivity for CD34.

CLINICAL PRESENTATION

The case is reported of a 60-year-old man with an anterior cranial fossa meningeal-based mass, which was resected. Histology showed some features in common with SFT (variable cellularity, spindled morphology, CD34 expression), but included an epithelioid element with cytokeratin and desmin immunopositivity, and lacked the characteristic vascular pattern of SFT. Histological features of meningioma were lacking. Recurrence of the tumour with extracranial extension 9 years later resulted in death of the patient. Histological examination revealed similar biphasic epithelioid and spindled CD34-immunopositive appearance to the earlier tumour, but in addition showed a high-grade element resembling olfactory neuroblastoma.

CONCLUSION

This case report is of a meningeal-based mesenchymal neoplasm with histological similarities to SFT. Its morphology and immunophenotype, however, are distinct from SFT and hence it is proposed that it is a newly described entity. In addition, recurrence of the tumour with a high-grade neuroblastic element has, to our knowledge, not previously been described in SFT.

摘要

背景

孤立性纤维瘤(SFT)是一种罕见的间叶性肿瘤,现在在许多部位都有描述,包括脑膜。颅内 SFT 在临床上和影像学上与脑膜瘤非常相似,并且与脑膜瘤一样,脑膜 SFT 的报告表明在完全切除后具有相对良性的行为。与脑膜瘤区分开来的 SFT 的组织病理学特征包括可变的细胞性、呈束状排列的梭形细胞、鹿角状血管和 CD34 的免疫阳性。

临床表现

报告了一例 60 岁男性,其额窦脑膜基肿块被切除。组织学显示与 SFT 有一些共同特征(可变的细胞性、梭形形态、CD34 表达),但包括上皮样成分,对细胞角蛋白和结蛋白呈免疫阳性,并且缺乏 SFT 的特征性血管模式。缺乏脑膜瘤的组织学特征。9 年后肿瘤复发并向颅外扩展导致患者死亡。组织学检查显示与早期肿瘤相似的双相上皮样和梭形 CD34 免疫阳性外观,但此外还显示出类似于嗅神经母细胞瘤的高级别成分。

结论

本病例报告是一种脑膜基间质性肿瘤,其组织学与 SFT 相似。然而,其形态和免疫表型与 SFT 明显不同,因此建议将其视为一种新描述的实体。此外,我们所知,在 SFT 中,肿瘤的复发性高级别神经母细胞成分以前没有被描述过。

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