CAJ | 학술논문

目的：探讨儿童累及下丘脑肿瘤的临床及影像学特征。方法收集40例累及下丘脑的肿瘤患者临床资料及影像学资料。其中30例经手术证实；10例下丘脑错构瘤经临床及影像学确诊。对患儿的临床症状进行分类总结；所有患儿的头颅 MR 图像由2名资深的神经放射医师进行分析。结果引起生长发育异常的肿物中,下丘脑旁错构瘤常见性早熟,呈等 T1等 T2且不强化的影像；颅咽管瘤常见发育迟缓,部分常伴视力障碍及少部分伴尿崩,为囊实性肿物,T1 WI 信号呈多样性,典型的可见 T1高信号,增强囊壁环状强化,实质部分不均质强化；生殖细胞瘤常见“三联征”———尿崩、发育迟缓及视力障碍,呈等 T1等 T2信号,常见多囊分隔或蜂窝状,明显强化。引起尿崩的肿物中,朗格罕氏组织细胞增生症(LCH)最常见,信号及位置与生殖细胞瘤相似,很少伴生长发育延迟。引起视力障碍,眼震及视力缺损以下丘脑区的视交叉/下丘脑星形细胞瘤最多见,症状出现早且单一,呈长 T1长 T2信号,本研究发现常伴“新月形”囊变；下丘脑内错构瘤以痴笑为主,影像同下丘脑旁错构瘤。结论累及下丘脑肿瘤临床表现具有一定内分泌异常特征,结合影像学特征可以准确对儿童累及下丘脑的病变作出定性诊断。
목적：탐토인동루급하구뇌종류적림상급영상학특정。방법수집40례루급하구뇌적종류환자림상자료급영상학자료。기중30례경수술증실；10례하구뇌착구류경림상급영상학학진。대환인적림상증상진행분류총결；소유환인적두로 MR 도상유2명자심적신경방사의사진행분석。결과인기생장발육이상적종물중,하구뇌방착구류상견성조숙,정등 T1등 T2차불강화적영상；로인관류상견발육지완,부분상반시력장애급소부분반뇨붕,위낭실성종물,T1 WI 신호정다양성,전형적가견 T1고신호,증강낭벽배상강화,실질부분불균질강화；생식세포류상견“삼련정”———뇨붕、발육지완급시력장애,정등 T1등 T2신호,상견다낭분격혹봉와상,명현강화。인기뇨붕적종물중,랑격한씨조직세포증생증(LCH)최상견,신호급위치여생식세포류상사,흔소반생장발육연지。인기시력장애,안진급시력결손이하구뇌구적시교차/하구뇌성형세포류최다견,증상출현조차단일,정장 T1장 T2신호,본연구발현상반“신월형”낭변；하구뇌내착구류이치소위주,영상동하구뇌방착구류。결론루급하구뇌종류림상표현구유일정내분비이상특정,결합영상학특정가이준학대인동루급하구뇌적병변작출정성진단。
Objective To evaluate the clinical and MRI features of tumor involving the hypothalamus in children.Methods 40 ca-ses of tumors involving the hypothalamus were included in this study.Of them,30 patients had surgery,with the final pathologic diagnosis.10 patients who were hypothalamus hamartomas based on clinical and MRI features.The patient’s clinical symptoms were classified.Two experienced neuroradiologists reviewed MRI features of tumors involving the hypothalamus.Results In tumors causing dysplasia,“beside the hypothalamic hamartoma”was often sexual precocity,which demonstrated a typical isointense on T1 or T2 weighted images,without enhancement.Craniopharyngioma presented growth retardation,sometimes accompanying with diabetes insipidus and visual impairment,which was a solid-cystic components with hyperintensity and calcification on T1 WI and ring-like en-hancement of cystic thin wall and heterogeneous enhancement of solid components on enhancement study.Germ cell tumors often had “triad”-diabetes insipidus,growth retardation and visual impairment,which showed isointense on T1 WI and T2 WI and signifi-cant enhancement,often associating with small cystic space or honeycomb.Dabetes insipidus caused by neoplasm often occurred in LCH,which signal and position were similar to that of germ cell tumors and rarely with growth retardation.Visual impairment often happened in astrocytomas that showed long T1 and long T2 signals with significant enhancement and“crescent-shaped ”cystic charac-teristic in this study.Hypothalamic hamartoma that lied in the mammillary body displayed giggle,which signal was as same as “be-side the hypothalamic hamartoma”.Conclusion Tumor involving the hypothalamus in pediatric shows some endocrine characteris-tics.Clinical findings in addition to the patient’s key MR imaging features at presentation,may be helpful in developing the differen-tial diagnosis for lesions involving the hypothalamic region.