Objective and importance: Four rare cases of central nervous system solitary fibrous tumors (C-SoFTs) are described. This tumor has not previously been reported in children or in deep cortical structures. Three of these tumors occurred in the posterior fossa. Only four cases in the posterior fossa have been described previously. Nine years after its debulking from the posterior fossa, one tumor disseminated to the spine, lung, and liver. Only one such aggressive C-SoFT has been described previously.
Clinical presentation: A 7-year-old child had had 1 year of right-sided weakness at presentation. Magnetic resonance imaging scans revealed a left basal ganglia lesion. A 49-year-old woman and a 30-year-old man presented after experiencing headaches for months. Magnetic resonance imaging scans in these patients revealed a tumor in the fourth ventricle and right jugular foramen, respectively. A 55-year-old man had spinal, liver, and lung dissemination of a previous posterior fossa tumor at presentation.
Intervention and technique: All four patients underwent craniotomy for resection or subtotal removal of the tumor. Intraoperative observations noted solid well-encapsulated tumors. Immunohistochemistry confirmed C-SoFTs in all four cases.
Conclusion: C-SoFTs are rare central nervous system, typically dural-based, tumors. They frequently are overlooked in the differential diagnosis of solid central nervous system tumors. Our findings suggest that these tumors can occur at any age and in most locations, regardless of proximity to the meninges (basal ganglia and ventricle), suggesting that the cells of origin are not meningothelial, but rather the mesenchyme of the cerebral vasculature. T1- and T2-weighted magnetic resonance imaging was notable for areas of hypointensity and of hyperintensity best described as patchy or a ying-yang appearance in all cases. Delayed extracranial metastasis may be noted.