病例73——女，6岁病史不详（有病理）

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手机分享病例，部分图像模糊。感谢张高立老师（ 天水市一 放射）。

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Solitary fibrous tumors (SFT) were originally described as primary neoplasms of the visceral pleura, well-encapsulated spindle-cell tumors that only became symptomatic by mass effect. However, they were found to have a mesenchymal origin and were seen in numerous extrapleural sites, including within the central nervous system (intraspinal, intracranial, and along cranial nerves).

孤立性纤维瘤（SFT）最初被认为是脏层胸膜的原发性肿瘤，包膜完整的梭形细胞肿瘤，有占位效应时产生相应症状。然而，它常被发现为间叶细胞起源，发生在许多胸膜以外的部位，包括中枢神经系统内（椎管内、颅内、颅神经走行区）。

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Hemangiopericytomas (HPC) were described as a distinct entity, classified as a subtype of meningiomas, thought to arise from smooth muscle pericytes of dural capillaries. However, like solitary fibrous tumors, they were found to arise from fibroblasts and share the same molecular genetics as SFTs. In fact, SFTs and HPCs were combined into the same entity in the 2016 World Health Organization (WHO) classification of central nervous system tumors (with hemangiopericytomas now being an obsolete term). SFT/HPC is a highly cellular and vascular tumor (WHO grades I to III) that often has systemic metastases at diagnosis (liver, lung, and bones). Classic histologic features include a “staghorn” branching stromal vascular pattern.

血管外皮细胞瘤（HPC）曾被认为是***存在的脑膜瘤的一种亚型，起源于硬脑膜毛细血管平滑肌的周细胞。然而，与SFT一样，它被发现起源于纤维母细胞，具有与SFT相同的分子遗传学。事实上，CNS肿瘤2016版WHO分类中，SFTs和HPCs被认为是同一种肿瘤（HPCs这个术语已经过时）。孤立性纤维瘤是一种富含细胞和血管的肿瘤（WHO I~III），可出现全身转移（肝、肺及骨）。组织学典型表现为间质血管呈“鹿角样”分支形状。

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感谢楼主无私分享

韩**心

好贴，好例子