Demaerel Philippe, Van Gool Stefaan
Department of Radiology , University Hospital K.U.Leuven, Herestraat 49, 3000, Leuven, Belgium.
Neuroradiology. 2008 Jan;50(1):85-92. doi: 10.1007/s00234-007-0323-0. Epub 2007 Nov 6.
Langerhans cell histiocytosis, previously known as histiocytosis X, is a complex disease consisting of three entities that are all characterized by a proliferation of the Langerhans cell. The clinical course is variable and ranges from a solitary lytic bone or skin lesion with complete remission to a multisystem disorder with possible lethal outcome. The clinical suspicion can be increased based on radiological findings that are important criteria in defining the extent of the disease involvement. A biopsy is often necessary for establishing the final diagnosis. The lytic craniofacial bone lesions are the most common craniospinal abnormality in Langerhans cell histiocytosis. Abnormalities in the hypothalamic-pituitary region are the most frequent manifestations, often accompanied with diabetes insipidus as the presenting symptom. A range of different central nervous system abnormalities can be recognized. It is important to be able to recognize the extensive spectrum of neuroradiological abnormalities in order to arrive at the diagnosis. Neuroimaging plays an even more important role in assessing the response to treatment or possible relapse.
朗格汉斯细胞组织细胞增多症，以前称为组织细胞增多症X，是一种复杂的疾病，由三个实体组成，其共同特征是朗格汉斯细胞增殖。临床病程多变，从伴有完全缓解的孤立性溶骨性骨或皮肤病变到可能导致致命后果的多系统疾病。基于影像学检查结果可增加临床怀疑，这些结果是定义疾病累及范围的重要标准。通常需要进行活检以确立最终诊断。溶骨性颅面骨病变是朗格汉斯细胞组织细胞增多症最常见的颅脊柱异常。下丘脑 - 垂体区域异常是最常见的表现，常伴有尿崩症作为首发症状。可以识别一系列不同的中枢神经系统异常。能够识别广泛的神经放射学异常对于做出诊断很重要。神经影像学在评估治疗反应或可能的复发方面发挥着更重要的作用。
