Grois N, Tsunematsu Y, Barkovich A J, Favara B E
St. Anna Children's Hospital, Vienna, Austria.
Br J Cancer Suppl. 1994 Sep;23:S24-8.
Diabetes insipidus and anterior pituitary dysfunction, are familiar central nervous system (CNS) complications of Langerhans cell histiocytosis (LCH) but the pathophysiology and biological behaviour of other forms of CNS involvement in LCH are poorly understood. In an attempt to improve our understanding of these rare complications, we studied 23 patients with LCH in whom neuroradiological abnormalities, with or without neurological dysfunction other than diabetes insipidus, developed during the course of disease. Neuroradiological abnormalities were of three basic types (a) poorly-defined changes in white matter, (b) well-defined changes in white and grey matter and (c) extra-parenchymal "tumoural" masses. There was a profusion of associated neurological signs and symptoms in most cases but some patients were asymptomatic. The neuropathological features were complex but infiltration of the CNS by histiocytes with xanthomatous change, particularly prominent in mass lesions, was common in the 13 cases in which biopsies were done. Patients with lytic lesions of the skull and diabetes insipidus are evidently most at risk of developing these rare manifestations of LCH. Therapeutic questions could not be answered from this study because no standard treatment had been given and outcome varied widely.
尿崩症和垂体前叶功能障碍是朗格汉斯细胞组织细胞增多症（LCH）常见的中枢神经系统（CNS）并发症，但对于LCH累及CNS的其他形式的病理生理学和生物学行为了解甚少。为了更好地理解这些罕见并发症，我们研究了23例LCH患者，这些患者在疾病过程中出现了神经放射学异常，无论有无除尿崩症之外的神经功能障碍。神经放射学异常有三种基本类型：（a）白质内界限不清的改变；（b）白质和灰质内界限清晰的改变；（c）脑实质外“肿瘤样”肿块。大多数病例伴有大量相关的神经体征和症状，但有些患者无症状。神经病理学特征复杂，但在13例行活检的病例中，常见组织细胞浸润CNS并伴有黄色瘤样改变，在肿块病变中尤为明显。有颅骨溶骨性病变和尿崩症的患者显然最易发生LCH的这些罕见表现。由于未给予标准治疗且结局差异很大，本研究无法回答治疗问题。
