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散发性和 NF1 相关神经纤维瘤中的多核花状巨细胞:94 例临床病理研究。

Multinucleated floret-like giant cells in sporadic and NF1-associated neurofibromas: a clinicopathologic study of 94 cases.

机构信息

Dipartimento G.F. Ingrassia, Policlinico Universitario G. Rodolico, Anatomia Patologica, Università di Catania, Via S. Sofia 87, Catania, Italy.

出版信息

Virchows Arch. 2010 Jan;456(1):71-6. doi: 10.1007/s00428-009-0859-y. Epub 2009 Nov 25.

Abstract

Multinucleated floret-like giant cells (MNFGCs), similar to those commonly observed in pleomorphic lipoma and giant cell fibroblastoma, have been occasionally reported in gynecomastia and neurofibromas from patients affected by neurofibromatosis type 1 (NF1). Accordingly, it has been suggested that their detection, especially in an otherwise typical neurofibroma, could be a morphological clue to diagnosis of NF1. The aim of the present study was the identification of MNFGCs in a large series (94 cases) of sporadic and NF1-associated neurofibromas, to assess if their presence may indeed be a morphological marker of NF1. Numerous MNFGCs, namely, those that were easily apparent at low magnification (x50 and x100), were identified only in 5.3% of cases. In 18.1% of cases, a low number of these cells could be observed but only after a careful search, especially at higher magnification (x200 and x400). Immunohistochemically, all MNFGCs were stained with vimentin and CD34, but not with S-100 protein. Interestingly, there was no statistically significant correlation between MNFGCs (presence or absence) and NF1 (p = 0.73), gender (p = 0.59), age (p = 0.43), and site of tumor (cutaneous vs deep-seated soft tissue; p = 0.27). Our clinicopathologic findings suggest that MNFGCs in an otherwise typical neurofibroma are not a reliable marker of NF1, likely representing a morphological reactive change of the indigenous dermal or endoneurial fibroblasts or dendritic cells in response to unknown microenvironmental stimuli.

摘要

多核花状巨细胞(MNFGCs)类似于多形性脂肪瘤和巨细胞纤维母细胞瘤中常见的多核花状巨细胞,偶尔在 1 型神经纤维瘤病(NF1)患者的乳腺增生和神经纤维瘤中也有报道。因此,有人认为,这些细胞的检测,尤其是在其他方面典型的神经纤维瘤中,可能是 NF1 诊断的形态学线索。本研究的目的是在一系列散发性和 NF1 相关神经纤维瘤(94 例)中鉴定 MNFGCs,以评估其存在是否确实是 NF1 的形态学标志物。只有 5.3%的病例中很容易在低倍镜(x50 和 x100)下观察到大量的 MNFGCs。在 18.1%的病例中,这些细胞的数量较少,但只有经过仔细观察,尤其是在高倍镜(x200 和 x400)下才能观察到。免疫组化染色显示,所有 MNFGCs 均表达波形蛋白和 CD34,但不表达 S-100 蛋白。有趣的是,MNFGCs(存在或不存在)与 NF1(p=0.73)、性别(p=0.59)、年龄(p=0.43)和肿瘤部位(皮肤与深部软组织;p=0.27)之间无统计学显著相关性。我们的临床病理发现表明,在其他方面典型的神经纤维瘤中存在 MNFGCs 不是 NF1 的可靠标志物,可能代表对未知微环境刺激的反应性改变,这些改变源自真皮或神经内膜固有成纤维细胞或树突状细胞。

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