摘要：
目的 探讨儿童鞍区占位性病变的临床及影像学特征.方法 回顾性分析112例经病理证实的儿童鞍区占位性病变的临床及影像学资料.结果 颅咽管瘤37例,临床主要表现为颅压增高(57%)和尿崩症(11%),影像表现主要为伴有钙化的囊性肿瘤(81%);胶质瘤18例,主要临床表现为视力受损,其中13例为毛细胞星形细胞瘤(PA),影像学表现为显著强化的实性肿瘤(94%);生殖源性肿瘤16例,75%的患者伴有尿崩症,其中13例为生殖细胞瘤,表现为等高密度肿块,中重度强化,部分DWI呈等高信号;错构瘤7例,86%的患者伴痴笑样癫痫,影像学表现为下丘脑乳头体区不强化的等信号肿块;朗格汉斯细胞组织细胞增生症(LCH)5例,80%的患者伴尿崩症,影像学表现为垂体柄增粗伴神经垂体T1WI高信号消失;垂体瘤3例,Rathke囊肿23例,蛛网膜囊肿3例,大多表现为头痛等.结论 儿童鞍区肿瘤的临床及影像学表现各有特点,两者结合有助于提高诊断的正确率,为临床进一步处理提供依据.%Objective To evaluate the clinical and imaging features of the sellar region lesions in children.Methods The clinical and imaging features of 112 cases with sellar region lesions were analyzed retrospectively,which were confirmed by pathology.Results 37 cases were craniopharyngiomas, which had two main symptoms of intracranial hypertension(57%)and diabetes insipidus(11%).On imaging it demonstrated as a calcified cystic tumor(81%).18 cases were gliomas, the main clinical feature of which was decreased visual acuity,13 of them were pilocytic astrocytoma, which manifested as a solid tumor with significantly enhancement(94%).16 cases were germ cell tumors,the main complaint was diabetes insipidus (75%),13 of them were germinoma, which showed iso-high density on CT and moderate enhancement after administration of contrast.And it showed high signal intensity on DWI.7 cases were hamartoma,86% patients of which showed gelasmus epilepsy,the imaging showed iso-signal masses with no enhacement in the hypothalamic papillary region.5 cases were LCH,80% of which had the complaint of diabetes insipidus,imaging findings manifested as thinkened pituitary stalk and loss of hyperintensity of posterior pituitary on T1WI.3 cases were pituitary tumors.23 cases were Rathke's cleft cysts,3 cases were arachnoid cyst.Most of the patients presented with headache.Conclusion The clinical and imaging features shows some specific features, which is helpful to improve the correct rate of diagnosis and provide the basis for further treatment.
