朗格汉斯细胞组织细胞增生症的影像学诊断及病理学特征  

Langerhans cell histiocytosis:imaging and histopathologic features

作　　者：邢菲 孙娜娜[1] 刘希胜[1] XING Fei;SUN Nana;LIU Xisheng(Department of Radiology ,the First Affiliated Hospital of Nanjing Medical University,Nanjing 210029,China)

机构地区：[1]南京医科大学第一附属医院江苏省人民医院放射科,江苏南京210029

出　　处：《实用放射学杂志》2019年第8期1294-1298,共5页Journal of Practical Radiology

摘　　要：目的探讨朗格汉斯细胞组织细胞增生症(LCH)的影像学表现及其病理学特征,提高对本病的认识及诊断水平。方法回顾性分析经病理确诊的31例LCH的临床资料、影像资料及其病理免疫组化特征。结果 31例LCH发生于骨骼22例(颅骨11例,眼眶1例,椎体4例,肋骨1例,上肢骨 2例,下肢骨 3例),垂体2例,肝脏2例,肺2例,胸腺1例,甲状腺1例,淋巴结1例。本组病例发生于骨骼较多见,主要表现为局部骨质破坏伴软组织肿块形成;发生于垂体表现为垂体增大伴明显不均匀强化,部分病例神经垂体缩小;发生于肝脏表现为肝脏弥漫多发低密度结节;发生于肺表现为两肺弥漫多发囊状影及斑片高密度影;发生于胸腺表现为前纵隔软组织密度肿块;发生于甲状腺表现为两侧叶弥漫肿大密度减低且不均匀;发生于淋巴结表现为淋巴结肿大。免疫组化示:CD1a、S-100、CD68、Ki-67呈阳性。结论 LCH发生于骨骼多见,表现为局部骨质破坏伴软组织肿块形成;发生于肝脏、肺及甲状腺多表现为弥漫性病变;其余部位LCH无明显特异性影像表现;免疫组化CD1a、S-100阳性有重要诊断意义。Objective To analyze the imaging findings and pathologic features to improve the diagnose ability of Langerhans cell histiocytosis (LCH).Methods The clinical data, imaging findings and pathologic features of 31 cases with LCH confirmed by pathology were analyzed retrospectively.Results The 31 cases of LCH were involved in bone (22 cases including 11 cases in skull, 1 case in orbit,4 cases in vertebral body, 1 case in ribs,2 cases in upper limb bone, 3 cases in lower limb bone), pituitary (2 cases), liver (2 cases), lung (2 cases),thymus (1 case).thyroid (1 case) and lymph nodes (1 case).Most of the LCH lesions located in bone,and showed local bone destruction with soft tissue masses.LCH lesions in pituitary showed the pituitary was larger and significant heterogeneously enhanced, but 1 case of LCH showed the neurohypophysis was smaller.LCH lesions in liver showed diffuse multiple low-density nodules.LCH lesions in lung showed diffuse multiple cysts and high-density shadows in both lungs.LCH lesion in thymus showed a soft tissue density mass in the anterior mediastinum. LCH lesions in thyroid showed enlarged thyroid with heterogeneous low-density. LCH lesions in lymph nodes showed lymphadenopathy.The positive expression rate of immunohistochemical indexes were: CDla, S-100, CD68 and Ki-67.Conclusion LCH lesions are frequently found in bone,showing local bone destruction with soft tissue mass formation;LCH lesions in the liver, lung and thyroid showed diffuse multiple lesions;the other LCH lesions has no obvious characteristic imaging manifestation;CD1a and S-100 positive expressions are important for LCH.

关 键 词：朗格汉斯细胞组织细胞增生症 计算机体层成像 磁共振成像 

分 类 号：R816[医药卫生—放射医学] R814.42[医药卫生—临床医学]