Murthy Jagarlapudi M K, Challa Sundaram, Parida Subhendu, Murthy Tenneti V R K, Basha Paspala Syed Ameer
Department of Neurology, Radiology and Neurosurgery, CARE Institute of Neurosciences, CARE Hospitals, Banjara Hills, Hyderabad, Telangana, India.
Department of Pathology, Basavatarakam Indo American Cancer Hospital and Research Institute, Hyderabad, Telangana, India.
Neurol India. 2021 Mar-Apr;69(2):451-456. doi: 10.4103/0028-3886.314536.
Tumor-like mass lesion is a rare subtype of primary angiitis of the central nervous system (ML-PACNS). This report describes six patients of histologically verified ML-PACNS. The mean age was 44.5 years (range 25-68) and four were males. Presenting symptoms headache (5), focal neurologic deficits (5), and seizures (4). On magnetic resonance imaging (MRI) the lesion was unifocal in two and multifocal in four patients. Consistent radiological findings were mass lesions with heterointense internal morphology with areas of diffusion restriction (DWI), and variable post-contrast enhancement. Pathologically vasculitis was classified as: Granulomatous in one, necrotizing in two and lymphocytic in three. There were two deaths. In conclusion patients with ML-PACNS are likely to be younger and more likely to present with seizures. MRI lesions with hetrointense internal morphology with areas of DWI was a consistent finding and may be a clue for the diagnosis of ML-PACNS.
肿瘤样肿块病变是中枢神经系统原发性血管炎（ML-PACNS）的一种罕见亚型。本报告描述了6例经组织学证实的ML-PACNS患者。平均年龄为44.5岁（范围25-68岁），4例为男性。主要症状为头痛（5例）、局灶性神经功能缺损（5例）和癫痫发作（4例）。在磁共振成像（MRI）上，2例患者的病变为单灶性，4例为多灶性。一致的影像学表现为具有内部形态不均匀强化且伴有弥散受限区域（DWI）的肿块病变以及不同程度的增强后强化。病理上血管炎分类为：肉芽肿性1例，坏死性2例，淋巴细胞性3例。有2例死亡。总之，ML-PACNS患者可能更年轻，且更易出现癫痫发作。具有内部形态不均匀强化且伴有DWI区域的MRI病变是一个一致的表现，可能是诊断ML-PACNS的线索。
