中枢神经系统孤立性幼年性黄色肉芽肿1例并文献复习

Isolated juvenile xanthogranuloma of central nervous system:a case report and literature review

目的分析中枢神经系统孤立性幼年性黄色肉芽肿(JXG)临床及影像学特点,以提高对该病的认识。方法回顾性分析1例经手术病理证实的左颞枕部孤立性JXG的CT和MRI表现、临床特征,并复习相关文献。结果CT示颞枕部膨胀性稍高密度软组织肿块,伴点条状钙化,局部颅骨破坏,增强扫描后呈明显强化。MRI示肿块呈结节融合状,T_(1)WI呈等信号,T_(2)WI呈等低混杂信号,液体衰减反转恢复(FLAIR)上呈等低信号,扩散加权成像(DWI)上未见扩散受限。结论中枢神经系统孤立性JXG临床罕见,影像学表现具有一定特征性,认识这些特征有利于诊断。

Objective To investigate the clinical and imaging features of isolated juvenile xanthogranuloma(JXG)of the central nervous system,with the goal of enhancing our understanding of this disease.Methods The CT and MRI findings,along with the clinical features of a case of isolated JXG in the left temporo-occipital region,confirmed by surgery and pathology,were retrospectively analyzed.Relevant literature was also reviewed.Results CT revealed a dilatative,slightly hyper-dense soft tissue mass in the temporo-occipital region,accompanied by punctate and strip calcification.The mass destroyed the local skull and enhanced homogenously after the injection of contrast agent.MRI showed that the mass was nodular and fused,and exhibited iso-intensity on T_(1)WI,heterogeneous iso-to hypo-intensity on T_(2)WI,iso-to hypointensity on FLAIR,and no restriction on DWI.Conclusion Isolated JXG of the central nervous system is very rare,and the imaging manifestations of isolated JXG of the central nervous system are characteristic.A full understanding of these features is helpful for diagnosis.