下丘脑错构瘤9例临床病理探讨  

Hypothalamic hamarhoma:a clinicopathological analysis of 9 cases

作　　者：桂秋萍[1] 袁峥[2] 宋欣[1] 

机构地区：[1]解放军总医院病理科,北京100853 [2]中国医学科学院北京协和医学院肿瘤医院肿瘤研究所病理科,北京100021

出　　处：《诊断病理学杂志》2011年第2期106-109,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨下丘脑错构瘤的临床病理特征。方法收集下丘脑错构瘤手术病例9例,进行HE及免疫组化染色,并结合文献讨论。临床上4例表现为性早熟,1例为发育迟缓及视力障碍,1例表现为头痛,3例有痴笑性癫病史。所有肿瘤均位于下丘脑部位,影像学上均未有增强改变。结果 9例患者中男性3例,女性6例,年龄2～18岁,平均7.5岁。组织学上,病变区为大致正常的神经元及胶质纤维组织,但在细胞种类及分布上有所不同;大部分病例呈皮层样结构,由小神经元及星形细胞构成;部分见结节状小神经元聚集,部分为神经元弥散分布于胶质纤维中。其中2例由大神经节细胞组成,部分呈灶性聚集,周围见少突胶质细胞呈卫星包绕。胶质成分主要为星形细胞,偶见少突胶质样细胞;2例合并鞍上脂肪瘤。结论下丘脑错构瘤是一种少见的先天性脑发育异常,为一良性病变,单纯手术切除即可治愈。病理诊断时极易误诊为正常的脑组织。Objective To study the clinicopathologic features of hypothalamic hamartomas.Methods Hypothalamic hamartoma tissue specimens for histological study were obtained from 9 patients who had undergone surgical resection at our hospital between 2000 and 2010 years.Their mean age at surgery was 7.5 years（range,2 ～ 18 years）,the three patients had gelastic（laughing） epilepsy,the four patients had sexual precosity,one was growth slowing and visual acuity disturbance,and other was suffered from the headache.All hamartoma located in the hypothalamus,without enhancement in the MR image.Routinely processed,4 μm paraffin-embedded sections from all 9 cases were stained with hematoxylin and eosin,and immunohistochemistry.Results Microscopically,the all HHs were composed of abnormally distributed but cytologically normal neurons and glia,but there were differences in the cytology and distribution of cells.In most cases,the distribution just like cortex tissue,various sized nodules of small neurons predominated,with diffusely scattered small neurons and astrocytes between the nodules.Only two cases were composed of larger ganglion cells.Some areas demonstrated typical clusters of large ganglion cells,occasionally the oligodendrocyte satellite cells were seen around the ganglion neurons.The glial cells were usually astrocytes,occasionally oligodendrocytes.The two cases were associated with suprasellar lipoma.Conclusions Hypothalamic hamartoma is rare lesions of the congenital encephalodysplasia,and the mass is composed of ectopia brain tissue and located abnormal place.The HH may be misdiagnosed as the normal cortex sometime and it can be cured by operation alone.

关 键 词：中枢神经系统 下丘脑错构瘤 病理学 

分 类 号：R739.41[医药卫生—肿瘤]