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。
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 tu
