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目的:探讨原发性颅内Rosai Dorfman病患者的临床病理特征,以提高对该病的认识及诊断水平。方法:分析1例原发性颅内Rosai Dorfman病患者的临床表现、影像学特点、病理形态特征及免疫组织化学表现,并结合文献进行探讨。结果:患者为中年男性,主要临床表现为渐行性加重的视物模糊。脑部MRI检查提示前颅底、筛窦、蝶窦、额窦、双侧上颌窦、鼻腔内广泛占位,伴双侧视神经入眶段侵犯、双侧额叶及基底节区水肿灶;病理示组织细胞增生,细胞质内可见吞噬的淋巴细胞和其他细胞成分;免疫组化显示组织细胞S-100(+)、PGM-1(+)、Kappa(+)、Lamda(+),MIB-1(<1%,+)。结论:原发性颅内Rosai Dorfman病患者的的临床及影像学表现均缺乏特征性,团片状异常信号影伴增强后病灶明显强化为其主要特点,明确诊断依赖于手术标本的病理诊断。
Objective: To investigate the clinicopathological features of patients with primary intracranial Rosai Dorfman disease in order to improve their understanding and diagnosis of the disease. Methods: The clinical manifestations, imaging features, pathological features and immunohistochemical findings of one patient with primary intracranial Rosai Dorfman disease were analyzed and analyzed in combination with the literature. Results: The patient was middle-aged male, the main clinical manifestations of progressive blurred vision blurred. Brain MRI showed that the anterior skull base, ethmoid sinus, sphenoid sinus, frontal sinus, bilateral maxillary sinus and nasal cavity were widely occupied, with bilateral optic nerve infiltrating into the orbital segment, bilateral frontal lobes and basal ganglia edema; pathology Tissue cell proliferation and cytoplasm showed phagocytic lymphocytes and other cellular components. Immunohistochemistry showed that S-100 (+), PGM-1 (+), Kappa (+), Lamda (+), MIB-1 (<1%, +). CONCLUSION: The clinical manifestations and imaging features of patients with primary intracranial Rosai Dorfman disease are characterized by a lack of characteristic features. The enhancement of the abnormal signal patch of the same group is obviously enhanced as the main characteristic. The definite diagnosis depends on the pathological diagnosis of the surgical specimens.