孤立性下丘脑-垂体轴郎格罕细胞组织细胞增生症的临床与磁共振成像表现  

Clinical manifestations and MRI signs of isolated Langerhans＇ cell histiocytosis in hypothalamus-pituitary axis

作　　者：李庆玲[1] 郭若汨[2] 温学花[3] 郭宇[1] 陈燕铭[1] 叶小鸣[1] 沈君[3] 

机构地区：[1]中山大学附属第三医院特诊医疗中心,广东广州510630 [2]中山大学附属第三医院放射科,广东广州510630 [3]中山大学孙逸仙纪念医院放射科

出　　处：《实用放射学杂志》2012年第10期1604-1606,1611,共4页Journal of Practical Radiology

摘　　要：目的分析孤立性下丘脑一垂体轴郎格罕细胞组织细胞增生症的临床与MRI特点，以提高对本病的认识。方法对本院确诊的12例孤立性下丘脑-垂体轴郎格罕细胞组织细胞增生症的MRI资料和临床资料进行回顾性分析。结果12例孤立性下丘脑一垂体轴郎格罕细胞组织细胞增生症患者均有不同程度的尿崩症表现。MRI显示7例垂体柄呈结节状增粗，2例垂体柄呈均匀性增粗，3例漏斗部呈结节状增粗，11例垂体后叶正常高信号消失。结论临床表现有尿崩症，MRI示垂体后叶正常高信号消失，垂体柄增粗，特别是结节状增粗，需考虑到本病的可能。Objective To analyze the clinical manifestations and MRI signs of isolated Langerhans＇ cell histiocytosis in the hypo- thalamus-pituitary axis, so that to improve the knowledge of this disease. Methods MR imaging and clinical data of 12 cases with isolated Langerhans＇ cell histiocytosis in the hypothalamus-pituitary axis were analyzed retrospectively. Results All cases appeared with varying degree performance of diabetes insipidus. MR imaging showed nodular thickening of the pituitary stalk in 7 cases, hom- ogeneous thickening of the pituitary stalk in 2 cases, nodular thickening of infundibulum in 3 cases and normal neurohypophysis dis- appeared in 11 cases. Conclusion When the normal neurohypophysis with high signal intensity disappeares and pituitary stalk he- comes thickening especially nodular thickening showed by MR imaging with the performance of diabetes insipidus, the possibility of isolated Langerhans＇ cell histiocytosis should be considered.

关 键 词：郎格罕细胞组织细胞增生症 下丘脑-垂体轴 磁共振成像 尿崩症 脑 

分 类 号：R584[医药卫生—内分泌] R445.2[医药卫生—内科学]