颅内多发窦组织细胞增生伴巨大淋巴结病1例及文献分析  

Intracranial multiple sinus histiocytosis with massive lymphadenopathy:A case report and literature analysis

作　　者：张娇 黄纯海 徐凤成 周立宇 ZHANG Jiao;HUANG Chun-Hai;XU Feng-Cheng;ZHOU Li-Yu(Jishou University School of Medicine,Jishou,Hunan,416000,China;Department of Neurosurgery,First Affiliated Hospital of Jishou University/Xiangxi Autonomous Prefecture People’s Hospital,Jishou,Hunan,416000,China;Centre for Clinical and Translational Medicine Research,Jishou University,Jishou,Hunan,416000,China)

机构地区：[1]吉首大学医学院,湖南吉首416000 [2]吉首大学第一附属医院/湘西自治州人民医院神经外科,湖南吉首416000 [3]吉首大学临床转化与医学研究中心,湖南吉首416000

出　　处：《国际神经病学神经外科学杂志》2022年第3期71-73,共3页Journal of International Neurology and Neurosurgery

基　　金：国家自然科学基金(81560414)。

摘　　要：窦组织细胞增生伴巨大淋巴结病(SHML)是一种少见的组织细胞增生性病变,主要特征是窦组织细胞增生伴淋巴结肿大,其临床表现与肿瘤的位置和大小有关,常表现为头痛、头晕、呕吐、视力下降、行走不稳,累及鞍区的SHML还可表现为垂体功能下降。影像学表现多为累及硬脑膜的实质性肿快,没有特异性,MRI常表现为T1WI等信号,T2WI低信号,增强扫描明显强化。该例患者MRI表现与SHML一致,存在甲状腺功能降低、皮质醇分泌不足,病变累及脑膜及周围组织,范围广,累及几乎整个静脉窦、鞍区、部分颅骨,既有结节状又有条索状硬膜强化。患者经过手术、激素与免疫治疗后,2个月随诊时患者的呼吸困难、头痛、头晕、行走不稳及影像学表现均较治疗前好转。Sinus histiocytosis with massive lymphadenopathy(SHML)is a rare histiocytic proliferative disorder and mainly characterized by sinus histiocytosis with lymph node enlargement.Its clinical manifestations are related to the location and size of the tumor,and often include headache,dizziness,vomiting,vision loss,unsteadiness in walking,and decreased pituitary function in SHML involving the saddle area.The imaging manifestation is mostly a substantial swelling involving the dura mater with no specificity,and the magnetic resonance imaging(MRI)often shows iso-signal T1WI,low signal T2WI,and obvious enhancement on enhancement scan.The MRI presentation in this case was consistent with that in conventional SHML,with the presence of reduced thyroid function,insufficient cortisol secretion,extensive lesions involving the meninges and surrounding tissues and involving the entire venous sinus,saddle area,and part of the skull,and both nodular and striated dural enhancement.After surgery,hormonotherapy,and immunotherapy,the patient’s dyspnea,headache,dizziness,unsteadiness in walking,and imaging manifestations were improved at 2-month follow-up compared with those before treatment.

关 键 词：中枢神经系统病变 窦组织细胞增生伴巨大淋巴结病 脑膜瘤 鞍区 IGG4 MRI 

分 类 号：R651.1[医药卫生—外科学]