儿童脑内多灶性朗格汉斯组织细胞增生症一例并文献复习  

Multifocal intraparenchymal Langerhans＇ cell histiocytosis in a child： case report and review of the literature

作　　者：仇波[1] 王勇[1] 程鹏[1] 李光宇[1] 李心国[1] 王运杰[1] 

机构地区：[1]中国医科大学第一医院神经外科,沈阳110001

出　　处：《中华神经外科杂志》2012年第10期1015-1018,共4页Chinese Journal of Neurosurgery

摘　　要：目的提高对儿童脑内朗格汉斯组织细胞增生症的临床特征、诊断及治疗的认识。方法报告1例4岁患儿脑内多灶性朗格汉斯组织细胞增生症的诊断及治疗过程，并复习相关文献。结果患儿以颅内压增高为首发症状，影像学检查示颅内多灶性病变，合并蛛网膜囊肿1处。经过一系列实验室检查以进行鉴别诊断后，行开颅手术切除右额叶责任病灶，术后病理证实为颅内朗格汉斯组织细胞增生症。术后患儿颅高压症状缓解，未切除病灶行化疗。6个月随访证实切除病灶部位无复发，未手术部位病灶无进展。结论有占位效应的幕上脑内多灶性朗格汉斯组织细胞增生症报道较少。对于占位效应明显的病灶，应首选切除，并辅以术后化疗，可望控制病变进展。Objective To improve the understanding of intraeranial Langerhans＇ cell histiocytosis （LCH） in children. Method A 4 - year - old boy diagnosed with muhifocal intraparenehymal LCH and the consequent procedure of treatments were reported, as well as some comparable literatures were reviewed. Results The patient complained firstly of nausea and vomiting with a progressing headache. Multifocal intracranial lesions with mass effect concomitant with an arachnoid cyst were found in imaging examination. After a series of laboratory examinations, the right frontal mass （ which caused symptoms of intracranial hypertension ） was surgically excised. Histological examinations confirmed the diagnosis of intracranial Langerhans＇ cell histiocytosis （ILCH）. The patient＇s intraeranial hypertension symptoms were alleviated and the remaining foci were treated by LCH - directed standard chemotherapy. At the 6 - month follow - up visit, no recurrence of the excised lesion was found, and no change in the size of other lesions was seen. Conclusion Supratentorial intracerebral lesions with mass effect and enhancement have rarely been described; in this report, the histological features of and therapeutic options for such a case are discussed. Surgery for the intraeranial lesions with mass effect followed with LCH - directed standard chemotherapy was recommended.

关 键 词：朗格汉斯组织细胞增生症 颅内多灶性病变 儿童 

分 类 号：R726.5[医药卫生—儿科]