History: A 27-year-old woman presents with progressive vertigo, ataxia, and headaches.


A head MRI scan was performed. Axial and sagittal T1-weighted, axial and coronal T1-weighted postcontrast, axial T2-weighted, and axial T2-weighted fluid-attenuated inversion-recovery (FLAIR) MR images are shown below.

行颅脑MRI扫描,轴位/矢状T1WI、轴位/冠状T1增强、T2WI、T2WI FLAIR图像如下所示。






There is an avidly enhancing lobular mass with multiple internal cystic components centered in the posterior fossa measuring up to 4.3 cm. The mass is associated with the dura as it extends across the left tentorium cerebelli into the supratentorial region. Additionally, there are cerebrospinal fluid clefts, which suggests that the lesion is extra-axial. The mass does not exhibit restricted diffusion. There is mass effect on the fourth ventricle, which is nearly completely affected. There is obstructive hydrocephalus of the third and lateral ventricles with evidence of transependymal CSF resorption. There is vasogenic edema in the bilateral cerebellar hemispheres and vermis.



Differential diagnosis

  • Meningioma

  • Solitary fibrous tumor of dura (meningeal hemangiopericytoma)

  • Medulloblastoma

  • Hemangioblastoma

  • Neurosarcoidosis

  • Dural metastases

  • Meningeal melanocytoma


  • 脑膜瘤

  • 硬脑膜孤立性纤维瘤(脑膜血管外皮细胞瘤)

  • 髓母细胞瘤

  • 血管母细胞瘤

  • 神经系统结节病

  • 硬脑膜转移瘤

  • 脑膜黑色素瘤

Diagnosis: Solitary fibrous tumor of dura (meningeal hemangiopericytoma)


Discussion  讨论

Solitary fibrous tumor of dura

(meningeal hemangiopericytoma)

Pathogenesis 发病机制

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).


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)曾被认为是独立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。

Epidemiology 流行病学

They account for less than 1% of intracranial tumors and are most often found in younger adults (ages 30 to 45) with a slightly higher prevalence in males.


Clinical presentation 临床表现

Symptoms are secondary to mass effect and include headaches, seizures, and focal neurologic dysfunction.


Diagnosis 诊断

Although imaging findings may distinguish an SFT/HPC from a meningioma, pathology is necessary to confirm the diagnosis, ideally by immunohistochemistry identifying NAB2-STAT6 fusion.


Imaging findings  影像表现

  • SFT/HPCs are solitary, lobulated, heterogeneous masses.

  • They are highly vascular and avidly enhancing.

  • They can be infratentorial and/or supratentorial in location.

  • Most common locations include the occipital region and may involve the falx, tentorium, or dural sinuses.

  • They are extra-axial but may have a narrow base of dural attachment.

  • On CT, there may be erosion of adjacent bone but no calcifications or hyperostosis.

  • On MRI, they will be isointense to gray matter on T1- and T2-weighted imaging, but mass effect on adjacent brain will display T2/FLAIR hyperintensity.

  • On MR spectroscopy, there is often a myo-inositol peak.

  • Meningiomas are usually smoother, have a broader dural tail, cause hyperostosis, and have a central spoke-wheel vascular supply.


  • 单发肿物,有分叶,不均质;

  • 富血供,明显强化;

  • 幕下、幕上均可见

  • 常见发病部位:枕部,可累及大脑镰、小脑幕及硬脑膜窦;

  • 脑外病变,窄基底与硬脑膜相接;

  • CT上可表现邻近骨质受侵,无钙化及骨质增生;

  • MRI T1WI、T2WI上肿瘤与灰质信号一致,占位效应可导致周围脑实质在T2/FLAIR上表现为高信号;

  • MRS上常可见肌醇峰;

  • 脑膜瘤表现为边缘光滑,宽基底及硬膜尾征,骨质增生,中心可见轮辐样供血血管。

Treatment and prognosis 治疗及预后

  • They require total surgical resection with preoperative catheter embolization.

  • Adjuvant radiotherapy helps reduce the rate of recurrence.

  • They have a high risk of local recurrence (50% to 90%) and metastases many years later.

  • Patients have a median survival of three to six years without metastases and two years with metastases.

  • 术前行导管栓塞,而后手术完整切除;

  • 辅助放疗有助于降低复发率;

  • 局部复发率较高(50%-90%) ,数年后可发生转移;

  • 患者中位生存时间为6年(无转移)及2年(有转移)。




2021-05-11 原文



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