Objective: To explore the imaging features of meningeal Ewing sarcoma/peripheral primitive neuroectodermal tumours (pPNETs). Methods: The imaging features and pathological characteristics of eight cases of surgically and pathologically confirmed Ewing sarcoma/pPNET were analysed retrospectively in light of recent literature on the disease. Results: The peak age was between 10 and 20 years. The lesions tended to be spindle shaped and dural based, usually widely so. CT showed that the lesions had slightly uneven high density in five cases and iso-low mixed density in three cases; marked heterogeneous enhancement was seen in all cases after contrast injection. MRI of the lesions showed varying proportions of isointense and hypointense signal in all cases on unenhanced T 1 weighted imaging and varying proportions of isointense and hyperintense signal on T 2 weighted imaging. After contrast injection, marked heterogeneous enhancement was seen in all cases; three cases showed a short and nodular dural tail and five cases showed adjacent skull erosion and osteolysis. Pathological results included high cell density, haemorrhage and necrosis. The cells resembled lymphocytes and spindle cells with transparent cytoplasm. CD99 and vimentin were expressed by all tumour cells. Conclusion: Features of meningeal Ewing sarcoma/ pPNETs include peak incidence at 10-20 years of age, a broad connection to the meninges, a thick dural tail involved with tumour, skull and scalp erosion and early metastasis. Necrosis and cystic changes are the common histological findings. Advances in knowledge: The imaging features of meningeal Ewing sarcoma/pPNETs have not been reported. The study helps to identify meningeal Ewing sarcoma/ pPNETs and meningioma.Ewing sarcoma/peripheral primitive neuroectodermal tumours (pPNETs) are a group of highly malignant small round cell neoplasms, which include Ewing sarcoma, Askin tumour and pPNET. 1 We collected eight cases of meningeal Ewing sarcoma/pPNET confirmed by pathology and immunohistochemistry to review the imaging and pathological findings in order to deepen our understanding and improve the diagnosis of this disease, and to provide more accurate clinical information.
METHODS AND MATERIALSEight cases of meningeal Ewing sarcoma/pPNET were confirmed by surgery and pathology in the Second Hospital of Lanzhou University, Lanzhou, China, between November 2005 and December 2011. There were five males and three females aged between 7 and 23 years (mean age, 15 years). Presenting symptoms had been of 2 weeks to 18 months of duration. There were four cases presenting with headache, two cases with headache, dizziness and vomiting and two cases presenting with fever. One case had right proptosis, accompanied by visual disturbances. Two cases were accompanied by physical disabilities. Two cases had non-tender masses. Pre-operative imaging diagnoses were meningioma in four cases, metastatic tumour in one case, hemangiopericytoma in one case and skull osteosarcoma in one case.MRI scans were performed with a 1.0-T...