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成人朗格汉斯组织细胞增生症的影像学表现

作者:房国成 王金成

所属单位:江苏省泰兴市人民医院放射科(江苏 泰兴 225400)

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摘要

目的 分析成人朗格汉斯组织细 胞增生症累及不同系统的的影像表现,提 高对该病的诊断水平。方法 回顾性分析 经手术病理证实5例朗格汉斯组织细胞增 生症的影像学表现及临床资料。结果 5 例中2例单发,3例多发。累及骨质的病灶 多表现为穿凿样、溶骨性及膨胀性骨质破 坏,边缘清晰或模糊,部分伴有硬化边, 部分可见骨膜反应,MRI上表现为T1WI等 信号,T2WI稍高、高信号。发生于中枢 神经系统的变病1例现为三脑室漏斗部结 节,边缘清晰,MRI增强呈明显强化,另 一例表现为神经垂体T1WI信号减低。发生 于软组织的表现为软组织增厚,部分形成 软组织肿块。结论 成人朗格汉斯组织细 胞增生症是一种较罕见的疾病,可累及全 身各系统,可单发也可多发,病变表现各 异,无特异性的影像学表现,因此影像表 现结合临床、病理才能确诊。

Objective To improve the awareness of the imaging features of Langerhans cell histiocytosis(LCH) in different systems. Methods 5 patients of Langerhans cell histiocytosis comfirmed by surgey and histopathology were collected. All imagings were retrospectively analyzed. Results Of these 5 cases, 2 were solitary lesion, and 3 cases were multiple lesions. Involvement of bone lesions show the punched out, soluble osseous bone destruction, and swelling bone destruction, with clear or fuzzy edge, some lesions accompanied by harden edge, and some lesions accompanied with periosteal reaction.1 Lesion occurred in the central nervous system which located at the third ventricle infundibulum, and manifested as a nodule which had a clear edge, and showed significantly enhancement on MRI .The lesion manifested as thickening of the soft tissue when occurred in soft tissue. Conclusion Langerhans cells histocytosis is a relatively rare disease and may involves the whole body, the lesion can be single or multiple, with no specific imaging findings. Therefore, the diagnosis of Langerhan's cell histiocytosis (LCH) is often difficult and delayed.

【关键词】格汉斯组织细胞增生症;计 算机体层成像;磁共振成像

【中图分类号】R733;R445.2;R445.3

【文献标识码】A

【DOI】 10.3969/j.issn.1672- 5131.2017.11.042

前言

朗格罕细胞组织细胞增生症(LCH)是以朗格罕细胞的肿瘤性增生为 病理表现的一组罕见疾病,多发生于10岁以下的儿童,成人较少见。 LCH可以从局限单个器官或孤立性病灶到多系统器官累及不等,最常累 及骨和皮肤,其他常见受累器官还包括肺、肝、脾、淋巴结、骨髓及 中枢神经系统等。本文回顾性分析5例经手术病理证实的成人LCH患者 的临床及CT、MRI资料,总结其影像特点,旨在增强对本病的认识,提 高术前诊断率。