Department of Pathology, Lapeyronie Hospital, 371 Avenue du doyen Gaston Giraud, 34295 Montpellier Cedex 5, France.
J Neurooncol. 2011 Jan;101(2):297-300. doi: 10.1007/s11060-010-0241-6. Epub 2010 Jun 6.
We report a case of solitary fibrous tumour (SFT) involving the posterior fossa in a fetus of 25 weeks' gestation. SFT is a rare mesenchymal neoplasm, arising in various locations including the meninges. After disclosure of severe ventriculomegaly and posterior fossa mass measuring 45 mm in diameter, termination of pregnancy was performed in accordance with French legislation. Our neuropathological study revealed a tumour covered by meninges, with severe compression of the cerebellum and the brain stem. Microscopically, the tumour was highly cellular, made of packed small fusiform cells with branching vasculature and consistent expression of CD34. No extraneurological lesion was noted. Except cysts and vascular malformations, posterior fossa tumours have been exceptionally reported in fetuses. SFT was distinguished from hemangiopericytoma. In spite of the fact these tumours share many similarities, some criteria such as the staining pattern for CD34 instead indicated a SFT. Histology was distinctive of hemangioblastoma and primitive neuroectodermal tumour. The prognosis of solitary fibrous tumour, which is usually a benign tumour, was there worsened by the precocity of the onset and the local invasion causing disruption of the cerebellum, compression of the brain stem and severe ventriculomegaly.
我们报告了一例 25 孕周胎儿后颅窝孤立性纤维瘤(SFT)。SFT 是一种罕见的间叶性肿瘤,可发生于脑膜等多种部位。在发现严重的脑室扩张和直径为 45 毫米的后颅窝肿块后,根据法国法律进行了终止妊娠。我们的神经病理学研究显示,肿瘤被脑膜覆盖,小脑和脑干严重受压。显微镜下,肿瘤细胞丰富,由排列紧密的梭形细胞组成,具有分支状的脉管系统,一致表达 CD34。未发现神经外病变。除了囊肿和血管畸形外,后颅窝肿瘤在胎儿中极为罕见。SFT 与血管外皮细胞瘤不同。尽管这些肿瘤有许多相似之处,但一些标准,如 CD34 的染色模式,表明这是一种 SFT。组织学上有血管母细胞瘤和原始神经外胚层肿瘤的特征。孤立性纤维瘤的预后通常为良性肿瘤,但由于发病早和局部浸润,导致小脑破坏、脑干受压和严重的脑室扩张,预后恶化。