摘要
目的初步探讨鞍区朗格汉斯细胞组织细胞增生症(LCH)的疾病特征。方法回顾性分析2018年6月至2020年8月首都医科大学三博脑科医院神经外科收治的12例鞍区LCH患者的临床资料。7例行脑立体定向活组织检查术,4例行开颅鞍区病变切除术,1例行右侧顶骨病变切除术。分析其临床、影像学及病理学特征。结果12例患者的年龄为(29.0±16.3)岁(3~54岁),成人占比为9/12;男女患者的比例为5∶7。所有患者均以多饮、多尿起病。病程为1个月至15年。甲状腺功能异常者占比为10/10,术前出现高钠血症者占比为7/12。术前影像学诊断出现误诊的比例为11/12。头颅MRI结果显示所有患者均出现垂体后叶生理性高信号的缺失以及双侧视束水肿。12例患者中,4例肺部CT结果显示双肺弥漫多发小透亮区改变。12例患者病理标本的HE染色结果显示体积较大、细胞质丰富的组织细胞样细胞增生;免疫组织化学染色结果显示,S-100、CD1a及Langerin蛋白均呈阳性表达。结论鞍区LCH患者常以多饮、多尿起病,常伴甲状腺功能异常、高钠血症,成人、儿童均可患病,易被误诊。头颅MRI检查常见垂体后叶生理性高信号的缺失以及双侧视束水肿,部分伴双肺特征性改变。病理学特征为组织细胞样细胞增生,S-100、CD1a及Langerin蛋白呈阳性。
Objective To explore the characteristics of Langerhans cell histiocytosis(LCH)in sellar region.Methods The clinical data,12 patients with LCH in sellar region admitted to the Department of Neurosurgery,Sanbo Brain Hospital,Capital Medical University from June 2018 to August 2020 were retrospectively analyzed.Among them,7 cases underwent stereotactic biopsy,4 cases underwent craniotomy with resection of the sellar region lesions,and 1 case underwent resection of the right parietal bone lesions.The clinical,imaging and pathological characteristics of those cases were analyzed.Results The age of the 12 patients was 29.0±16.3 years(3-54 years),the proportion of adults was 9/12,and the ratio of male to female patients was 5∶7.All patients started with polydipsia and polyuria.The course of the disease ranged from 1 month to 15 years.The proportion of patients with abnormal thyroid function was 10/10,and the proportion of patients with preoperative hypernatremia was 7/12.The ratio of misdiagnosis in preoperative imaging was 11/12.The head MRI results showed that all patients had a loss of physiological hyperintensity in the posterior pituitary gland and bilateral optic tract edema.Among the 12 patients,4 showed diffuse and multiple small translucent areas in both lungs on CT results.The HE staining results of the pathological specimens of 12 patients showed the proliferation of histiocyte-like cells with large volume and rich cytoplasm;the immunohistochemical staining results showed that S-100,CD1a and Langerin proteins were all positively expressed.Conclusions Patients with LCH in sellar region often start with polydipsia and polyuria,and are often accompanied by abnormal thyroid function and hypernatremia.Adults and children can suffer from the disease and are easily misdiagnosed.In cranial MRI,the loss of physiological hyperintensity in the posterior pituitary gland and the edema of the bilateral optic fascicles are common,some of which are accompanied by characteristic changes in both lungs.The pathological features are proliferation of histiocyte-like cell and positive expression of S-100,CD1a and Langerin proteins.
作者
雷霆
刘锐
吴陈兴
周佳俊
齐雪岭
段泽君
姚坤
张旭妃
高洁
李守巍
Lei Ting;Liu Rui;Wu Chenxing;Zhou Jiajun;Qi Xueling;Duan Zejun;Yao Kun;Zhang Xufei;Gao Jie;Li Shouwei(Department of Neurosurgery,Sanbo Brain Hospital,Capital Medical University,Beijing 100093,China)
出处
《中华神经外科杂志》
CSCD
北大核心
2021年第12期1246-1250,共5页
Chinese Journal of Neurosurgery
基金
北京市自然科学基金(7182076)。
