CAJ | 학술논문

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.
목적 제고대인동뇌내랑격한사조직세포증생증적림상특정、진단급치료적인식.방법 보고1례4세환인뇌내다조성랑격한사조직세포증생증적진단급치료과정,병복습상관문헌.결과 환인이로내압증고위수발증상,영상학검사시로내다조성병변,합병주망막낭종1처.경과일계렬실험실검사이진행감별진단후,행개로수술절제우액협책임병조,술후병리증실위로내랑격한사조직세포증생증.술후환인로고압증상완해,미절제병조행화료.6개월수방증실절제병조부위무복발,미수술부위병조무진전.결론 유점위효응적막상뇌내다조성랑격한사조직세포증생증보도교소.대우점위효응명현적병조,응수선절제,병보이술후화료,가망공제병변진전.
Objective To improve the understanding of intracranial Langerhans' cell histiocytosis (LCH) in children.Method A 4 - year - old boy diagnosed with multifocal intraparenchymal 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 intracranial 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 intracranial lesions with mass effect followed with LCH - directed standard chemotherapy was recommended.