背景:脑包虫病相对罕见,在病理诊断方面,区分脑囊型包虫病(CCE)和脑肺泡型包虫病(CAE)很重要。我们旨在描述CCE和CAE患者的不同临床病理特征。
方法:收集2012年1月1日至2023年6月30日新疆医科大学第一附属医院病理科确诊的脑包虫病患者27例。我们比较了患者的临床特征,MRI特征,CCE和CAE的病理表现。
结果:在27例脑包虫病中,CAE23例,CCE4例。CCE和CAE患者的临床表现主要包括头痛(21例,77.78%),肢体运动障碍(6例,22.22%),癫痫发作(4例,14.81%)和视觉障碍(2例,7.41%)。CAE病例的平均发病年龄为34.96±11.11岁,CCE病例为9.00±7.26年。所有CAE患者均在大脑和颅外器官中表现出多种受累,而所有CCE患者均在大脑中观察到孤立性病变,而3例CCE病例没有颅外受累。MRI中CCE的病变显示为单个孤立的圆形,与周围组织的界限很好,病变周围没有明显的水肿,而CAE病变表现为颅内多发病变,病变周围边缘模糊和水肿,在病变中可以观察到多个小囊泡。CAE病灶边缘可增强,CCE病灶无明显强化。CCE病灶为透明囊肿,壁约0.1cm。微观上,囊肿壁以嗜酸性角质层为特征,一侧是嗜碱性胚层细胞,有时可见为原脑结节。虽然CAE病变是结节表面粗糙且不均匀的结节结构,切开的切片是囊性和实性的;显微镜下,CAE病变有凝固性坏死区域,而原头淋巴结几乎看不见.由巨噬细胞组成的炎症细胞区域,淋巴细胞,上皮样细胞,浆细胞,嗜酸性粒细胞,在病变周围可以看到成纤维细胞。炎症细胞区域附近的脑组织可能显示细胞凋亡,变性,坏死,和细胞水肿,而离病变稍远一点的脑组织显示出正常的形态。
结论:脑包虫病的发病率较低,对于病理学家来说,包虫病的诊断以及CAE和CCE的鉴别诊断具有挑战性.掌握CAE和CCE的不同临床病理特征有助于病理学家做出准确的诊断。
BACKGROUND: Cerebral echinococcosis is relatively rare, and it is important to distinguish cerebral cystic echinococcosis (CCE) from cerebral alveolar echinococcosis (CAE) in terms of pathological diagnosis. We aim to describe the different clinicopathological features among patients with CCE and CAE.
METHODS: We collected 27 cases of cerebral echinococcosis which were diagnosed in the Department of Pathology of the First Affiliated Hospital of Xinjiang Medical University from January 1, 2012, to June 30, 2023. We compared the patients\' clinical characteristics, MRI features, and pathologic manifestations of CCE and CAE.
RESULTS: Among 27 cases of cerebral echinococcosis, 23 cases were CAE and 4 cases were CCE. The clinical manifestations of both CCE and CAE patients mainly included headache (21 patients, 77.78%), limb movement disorders (6 patients, 22.22%), epileptic seizures (4 patients, 14.81%) and visual disturbances (2 patients, 7.41%). The average onset age of CAE cases was 34.96 ± 11.11 years, which was 9.00 ± 7.26 years in CCE cases. All CAE patients presented with multiple involvements in the brain and extracranial organs while all CCE patients observed a solitary lesion in the brain and 3 CCE cases had no extracranial involvement. Lesions of CCE in MRI showed a single isolated circular, which was well demarcated from the surrounding tissues and with no obvious edema around the lesions, whereas CAE lesions presented as multiple intracranial lesions, with blurred edges and edema around the lesions, and multiple small vesicles could be observed in the lesions. The edge of CAE lesions could be enhanced, while CCE lesions have no obvious enhancement. CCE foci were clear cysts with a wall of about 0.1 cm. Microscopically, the walls of the cysts were characterized by an eosinophilic keratin layer, which was flanked on one side by basophilic germinal lamina cells, which were sometimes visible as protocephalic nodes. While the CAE lesion was a nodular structure with a rough and uneven nodule surface, and the cut section was cystic and solid; microscopically, the CAE lesion had areas of coagulative necrosis, and the proto-cephalic nodes were barely visible. Inflammatory cell areas consisting of macrophages, lymphocytes, epithelioid cells, plasma cells, eosinophils, and fibroblasts can be seen around the lesion. Brain tissues in the vicinity of the inflammatory cell areas may show apoptosis, degeneration, necrosis, and cellular edema, while brain tissues a little farther away from the lesion show a normal morphology.
CONCLUSIONS: With the low incidence of brain echinococcosis, the diagnosis of echinococcosis and the differential diagnosis of CAE and CCE are challenging for pathologists. Grasping the different clinical pathology characteristics of CAE and CCE is helpful for pathologists to make accurate diagnoses.