颅内巨淋巴结增生临床特征分析

Clinical features of intracranial giant lymph node hyperplasia

目的:探讨颅内巨淋巴结增生(又名Castleman病)的临床特征,以期提高对该病的认识及诊治水平。方法:回顾性分析2017年2月9日南方医科大学珠江医院神经外科收治的1例颅内混合型巨淋巴结增生患者的临床资料,并进行相关文献复习。以"Castleman病""颅内""中枢神经系统"为检索词检索万方数据库和中国期刊全文数据库,同时以"Castleman""Intracranial""central nervous system"为检索词检索PubMed、Embase、Ovid和Cochrane数据库,检索文献的发表时间为1980年1月1日—2017年1月31日。结果:1例32岁女性患者因头痛1个月入院。CT检查显示右侧颞顶部可见边界清晰但不规则的高密度影,增强扫描可见明显强化;右侧颞顶叶见大片水肿区域。实验室检查提示贫血和高免疫球蛋白血症。经手术完整切除颅内肿块。术后病理诊断为右侧颞顶部混合型巨淋巴结增生。3个月后复查颅脑MRI,未见病变残留和复发征象。文献检索共获得17篇相关文献,包含20例颅内巨淋巴结增生病例。包含本病例在内的21例颅内巨淋巴结增生患者中,男性7例,女性14例,中位年龄为47岁(范围:8~82岁)。颅内巨淋巴结增生均发生于硬脑膜或软脑膜,主要位于大脑凸面和大脑镰旁;主要临床表现为癫痫和头痛;影像学表现为脑表面可见边界清晰的肿块,并伴病灶周围水肿,增强扫描可见明显的均匀强化。结论:颅内巨淋巴结增生极为罕见。结合临床表现和实验室检查,有助于该病的诊断。

Objective： To investigate the clinical features of intracranial Castleman＇s disease, and to improve the understanding of the disease. Methods： The clinical features of one case of intracranial mixed type Castleman＇s disease, who was diagnosed in Department of Neurosurgery, Zhujiang Hospital, Southern Medical University in February 9, 201 7, were retrospectively analyzed, and the relevant literatures about intracranial Castleman＇s disease were reviewed. The PubMed, Embase, Ovid, Cochrane, Wanfang Database and China Journal Full-text Database were searched using the keywords including ＂Castleman＂, ＂lntracranial＂ and ＂Central nervous system＂ for related literatures published between January 1, 1980 and January 31,2017. Results： A 32-year old female was admitted because of headache for one month. CT examination showed an irregular and well-defined hyperdense lesion in the right temporo- parietal area with surrounding extensive edema and this mass was markedly contrast enhanced. Laboratory examination revealed anemia and hyperimmunoglobulinemia. The mass was removed completely by surgical resection. Postoperative pathology examination revealed mixed type Castleman＇s disease in the right temporo-parietal area. MRI was performed 3 months after the surgery. No residual tumor and recurrence were demonstrated. Seventeen related case reports were retrieved and 20 cases of intracranial Castleman＇s disease were reported. Among total 21 cases, there were 7 males and 14 females, ranging from 8 to 82 years old, and the median age was 47 years old. The mass arised from the dura matter or leptomeninges, and the most common sites of origin were located at the cerebral convexity and the parafalcine. The most common clinical features were seizures and headache. Radiographically, the previously reported cases presented as a homogeneously enhanced extra-axial well-defined mass with a marked peritumoral edema. Conclusion： Intracranial Castleman＇s disease is made in combination with clinical findings and a rare clinical condition. The diagnosis can be the laboratory examination.