中华内科杂志
中華內科雜誌
중화내과잡지
CHINESE JOURNAL OF INTERNAL MEDICINE
2015年
9期
758-762
,共5页
张亭亭%付永娟%卢德宏%李存江%朴月善
張亭亭%付永娟%盧德宏%李存江%樸月善
장정정%부영연%로덕굉%리존강%박월선
中枢神经系统%病理学%组织细胞增多症,非郎格尔汉斯细胞%罗塞-道夫曼病%播散性黄色瘤
中樞神經繫統%病理學%組織細胞增多癥,非郎格爾漢斯細胞%囉塞-道伕曼病%播散性黃色瘤
중추신경계통%병이학%조직세포증다증,비랑격이한사세포%라새-도부만병%파산성황색류
Central nervous system%Pathology%Histiocytosis,non-Langerhans-cell%Rosai-Dorfman disease%Xanthoma disseminatum
目的 研究中枢神经系统非朗格汉斯细胞组织细胞增生症的临床、病理及影像学特点,探讨其诊断及鉴别诊断要点.方法 收集首都医科大学宣武医院2010-2014年10例中枢神经系统非朗格汉斯细胞组织细胞增生症[罗塞-道夫曼病7例,播散性黄色瘤3例]的临床病理资料,分析其临床病史、影像学、病理组织学、免疫表型及预后特点.结果 7例罗塞-道夫曼患者中,男5例,女2例,平均年龄46.7岁;影像学表现为形态各异的占位性病变,大部分为硬脑/脊膜受累,仅1例病变单纯发生于脑实质内,与硬脑膜无关系;MRI示病灶表现为T1低或等信号影,T2低、等或稍高信号影,增强后可见强化;镜下于慢性炎性细胞浸润及纤维化的背景中可以看到多量组织细胞分布,部分组织细胞可见“伸入”现象,免疫组化显示组织细胞表达CD11c、CD68、MAC387及S-100蛋白,不表达CD1α,其中1例同时合并IgG4相关的慢性肥厚性硬脊膜炎;随访到6例罗塞-道夫曼病患者,5例恢复良好,1例死于该病.3例播散性黄色瘤患者均为女性,中位年龄20岁;影像学显示3例均为多发病变,累及颅内不同部位,MRI均表现为T1低或等信号,T2高信号,增强后可见强化;镜下可见大量泡沫样组织细胞增生,散在Touton多核巨细胞、淋巴细胞及嗜酸性粒细胞浸润,组织细胞表达CD68及CD11c,部分表达MAC387,不表达S-100及CD1α;随访到2例播散性黄色瘤患者,1例恢复良好,1例死于该病.结论 罗塞-道夫曼病及播散性黄色瘤都具有较为典型的组织学及免疫组化特点,但应该与朗格汉斯细胞组织细胞增生症及其他非朗格汉斯细胞组织细胞增生症鉴别,还应该与和各自形态学相似的其他疾病鉴别.由于这类疾病临床表现及影像学的不典型性,临床医生与病理医生需要进行良好有效的沟通及合作,以提高诊断正确率.
目的 研究中樞神經繫統非朗格漢斯細胞組織細胞增生癥的臨床、病理及影像學特點,探討其診斷及鑒彆診斷要點.方法 收集首都醫科大學宣武醫院2010-2014年10例中樞神經繫統非朗格漢斯細胞組織細胞增生癥[囉塞-道伕曼病7例,播散性黃色瘤3例]的臨床病理資料,分析其臨床病史、影像學、病理組織學、免疫錶型及預後特點.結果 7例囉塞-道伕曼患者中,男5例,女2例,平均年齡46.7歲;影像學錶現為形態各異的佔位性病變,大部分為硬腦/脊膜受纍,僅1例病變單純髮生于腦實質內,與硬腦膜無關繫;MRI示病竈錶現為T1低或等信號影,T2低、等或稍高信號影,增彊後可見彊化;鏡下于慢性炎性細胞浸潤及纖維化的揹景中可以看到多量組織細胞分佈,部分組織細胞可見“伸入”現象,免疫組化顯示組織細胞錶達CD11c、CD68、MAC387及S-100蛋白,不錶達CD1α,其中1例同時閤併IgG4相關的慢性肥厚性硬脊膜炎;隨訪到6例囉塞-道伕曼病患者,5例恢複良好,1例死于該病.3例播散性黃色瘤患者均為女性,中位年齡20歲;影像學顯示3例均為多髮病變,纍及顱內不同部位,MRI均錶現為T1低或等信號,T2高信號,增彊後可見彊化;鏡下可見大量泡沫樣組織細胞增生,散在Touton多覈巨細胞、淋巴細胞及嗜痠性粒細胞浸潤,組織細胞錶達CD68及CD11c,部分錶達MAC387,不錶達S-100及CD1α;隨訪到2例播散性黃色瘤患者,1例恢複良好,1例死于該病.結論 囉塞-道伕曼病及播散性黃色瘤都具有較為典型的組織學及免疫組化特點,但應該與朗格漢斯細胞組織細胞增生癥及其他非朗格漢斯細胞組織細胞增生癥鑒彆,還應該與和各自形態學相似的其他疾病鑒彆.由于這類疾病臨床錶現及影像學的不典型性,臨床醫生與病理醫生需要進行良好有效的溝通及閤作,以提高診斷正確率.
목적 연구중추신경계통비랑격한사세포조직세포증생증적림상、병리급영상학특점,탐토기진단급감별진단요점.방법 수집수도의과대학선무의원2010-2014년10례중추신경계통비랑격한사세포조직세포증생증[라새-도부만병7례,파산성황색류3례]적림상병리자료,분석기림상병사、영상학、병리조직학、면역표형급예후특점.결과 7례라새-도부만환자중,남5례,녀2례,평균년령46.7세;영상학표현위형태각이적점위성병변,대부분위경뇌/척막수루,부1례병변단순발생우뇌실질내,여경뇌막무관계;MRI시병조표현위T1저혹등신호영,T2저、등혹초고신호영,증강후가견강화;경하우만성염성세포침윤급섬유화적배경중가이간도다량조직세포분포,부분조직세포가견“신입”현상,면역조화현시조직세포표체CD11c、CD68、MAC387급S-100단백,불표체CD1α,기중1례동시합병IgG4상관적만성비후성경척막염;수방도6례라새-도부만병환자,5례회복량호,1례사우해병.3례파산성황색류환자균위녀성,중위년령20세;영상학현시3례균위다발병변,루급로내불동부위,MRI균표현위T1저혹등신호,T2고신호,증강후가견강화;경하가견대량포말양조직세포증생,산재Touton다핵거세포、림파세포급기산성립세포침윤,조직세포표체CD68급CD11c,부분표체MAC387,불표체S-100급CD1α;수방도2례파산성황색류환자,1례회복량호,1례사우해병.결론 라새-도부만병급파산성황색류도구유교위전형적조직학급면역조화특점,단응해여랑격한사세포조직세포증생증급기타비랑격한사세포조직세포증생증감별,환응해여화각자형태학상사적기타질병감별.유우저류질병림상표현급영상학적불전형성,림상의생여병리의생수요진행량호유효적구통급합작,이제고진단정학솔.
Objective To explore the clinicopathological features and imaging characteristics of non-Langerhans cell histiocytosis in central nerve system,thus to facilitate the diagnosis and differential diagnosis.Methods A total of ten cases were enrolled in the study,with seven cases of Rosai-Dorfman disease(RDD) and three cases of xanthoma disseminatum (XD).Data on the clinicopathological features,imaging,immunophenotype and prognosis were collected and analyzed.Results Seven patients with RDD,5 males and 2 females with the mean age of 46.7 years old,all presented as dural-based or intraparenchymal hypo-to isointense lesions on T1 and T2 with post-contrast enhancement.The polymorphous admixture of histiocytes,lymphocytes and plasma cells was observed in a fibrous stroma,with emperipolesis of some histiocytes.The immunohistostaining of CD11c,CD68,MAC387 and S-100 was positive in the histiocytes,while the staining of CD1α was negative.Five patients recovered after the operation,while one patient died of the disease.All the 3 XD patients were female,with the median age of 20.7 years old.All XD patients presented as multiple intraparenchymal hypointense lesions on T1 and hyperintense lesions on T2 with post-contrast enhancement.The infiltration of foam-like histiocytes,a few Touton giant cells,lymphocytes and eosnophils was observed in all XD patients.The immunohistostaining of CD68 and CD11c was positive in the histiocytes and that of MAC387 partly positive,while the staining of S-100 and CD1α was negative.One XD patient survived well,while another one died of the disease.Conclusions The diagnosis of RDD and XD should be based on their typical morphology and immunophenotype and should be differentiated from Langerhans cell histiocytosis and other non-Langerhans cell histiocytosis.Non-Langerhans cell histiocytosis in central nerve system often presents untypical clinical presentation and imaging features,thus the communication and cooperation between clinician and pathologist is needed.
