Bathla Girish, Kandemirli Sedat Giray, Gupta Sarika, Agarwal Amit
Department of Radiology University of Iowa, Iowa, United States.
Department of Pathology, University of Iowa, Iowa, United States.
Surg Neurol Int. 2020 Dec 22;11:463. doi: 10.25259/SNI_830_2020. eCollection 2020.
Sarcoidosis is an idiopathic, granulomatous, and multi-system inflammatory disorder that can also involve the central nervous system in the form of meningeal, parenchymal, or cranial nerve involvement. Imaging findings can be non-specific and may overlap with other inflammatory, infectious and neoplastic processes, and posing diagnostic challenges. Parenchymal involvement in neurosarcoidosis (NS) predominantly manifests as either non-enhancing white matter lesions or as enhancing parenchymal granulomas. Granulomas usually manifest as multiple solid lesions with nodular enhancement.
A 72-year-old man presented with right-eye visual field changes with the non-contrast head computed tomography showing a large cystic lesion in the left frontoparietal lobe. Subsequent contrast-enhanced magnetic resonance imaging study revealed a large cystic mass with irregular rim enhancement and mural nodule concerning for glial neoplasm. Cyst decompression with biopsy and histopathological analysis revealed gliosis and prominent perivascular granulomatous inflammation with mixed picture of CD4 and CD8-positive cells suggestive of sarcoidosis. Further subsequent work-up showed mediastinal and cervical lymphadenopathy which on biopsy showed non-necrotizing granulomatous inflammation, consistent with sarcoidosis.
Herein, we report unique imaging findings of a NS case manifesting as a solitary cystic intraparenchymal lesion with an enhancing nodular component, mimicking primary intra-cranial tumor. This appearance is highly atypical and rarely been reported earlier.
结节病是一种特发性、肉芽肿性多系统炎症性疾病,也可累及中枢神经系统,表现为脑膜、实质或颅神经受累。影像学表现可能不具有特异性,可能与其他炎症、感染和肿瘤性病变重叠,带来诊断挑战。神经结节病(NS)的实质受累主要表现为无强化的白质病变或强化的实质肉芽肿。肉芽肿通常表现为多个有结节状强化的实性病变。
一名72岁男性因右眼视野改变就诊,头部非增强计算机断层扫描显示左额顶叶有一个大的囊性病变。随后的对比增强磁共振成像研究显示一个大的囊性肿块,边缘不规则强化且有壁结节,怀疑为神经胶质瘤。囊肿减压活检及组织病理学分析显示胶质增生和显著的血管周围肉芽肿性炎症,CD4和CD8阳性细胞混合存在,提示结节病。进一步检查显示纵隔和颈部淋巴结肿大,活检显示非坏死性肉芽肿性炎症,与结节病一致。
在此,我们报告了一例NS病例的独特影像学表现,表现为孤立的脑实质内囊性病变,伴有强化的结节成分,类似原发性颅内肿瘤。这种表现非常不典型,此前很少有报道。