原发性颅内Rosai Dorfman病1例报道及文献复习

Primary intracranial Rosai Dorfman disease:report of a case and review of literature

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 clinicopathologic characteristics of primary intracranial Rosai Dorfman dis- ease （RDD） for improving the diagnosis criteria of RDD. Methods： Clinical symptoms, imaging features, pathological characteristics and immunohistochemieal phenotyping of a patient with intracranial primary RDD were analyzed and with literatures reviewed. Results- The main clinical manifestation of this middle aged male patient was blurred vision, which deteriorated gradually. MRI imaging showed the lesion was located at anterior skull base, ethmoid sinus, sphenoid sinus, frontal sinus, bilateral maxillary sinus and nasal cavity,with intraorbital optic nerve involved and edematous lesion of bilat- eral frontal lobe and basalganglia. Histological examination demonstrated numerous proliferative histiocytes and phagocy- tized lymphocytes and other cellular components within the cell cytoplasm.Immunohistochemical analysis showed that the histiocytes were positive for S-100, PGM-1, Kappa and Lamda, and MIB-1 was positive in less than 1%. Conclusions： Clinical and imaging features of primary intracranial Rosai Dorfman disease are not specific, and the main characteristic is the patchy abnormal signal imaging and significant enhancing of the lesion after enhancement. The definite diagnosis depends on pathological examination of the surgical specimen.