Cheng Lidong, Zhu Hongtao, Wang Jing, Wang Guanghui, Ma Xiaoyu, Zhao Kai, Wang Junwen, Shu Kai
Department of Neurosurgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Front Neurol. 2022 Jun 6;13:920505. doi: 10.3389/fneur.2022.920505. eCollection 2022.
Primary ventricular lymphoma (PVL) is an extremely rare and commonly misdiagnosed disease. Previous studies were predominantly case reports, and literature regarding the diagnosis and treatment of PVL is limited. Therefore, this study aimed to evaluate the characteristics of patients with PVL.
The data of patients with pathologically confirmed PVL were assessed. Epidemiological data, imaging findings, surgery, pathological results, and prognosis were retrospectively analyzed. A systematic review of relevant literature was also conducted.
A total of eight patients with PVL were identified. The main symptom was increased intracranial pressure. Radiographically, five patients had single lesion and three had multiple lesions; typical findings on magnetic resonance imaging included hypointensity on T1- and T2-weighted imaging, adjacent brain edema, and homogeneous enhancement on contrast-enhanced T1-weighted images. Preoperatively, six cases were misdiagnosed and two cases did not get a definite diagnosis. Craniotomy was performed on all patients, and four achieved gross total resection. Hydrocephalus was relieved after surgical resection in four patients. Pathology revealed diffuse large B-cell lymphoma in all patients. Only one patient had a severe complication. A total of three patients received concomitant adjuvant treatment, whereas five patients refused any adjuvant therapy. At the time of follow-up, the median survival time of patients was 15 months.
Primary ventricular lymphoma mainly presented with symptoms of increased intracranial pressure and had several imaging characteristics for the diagnosis, but the condition still tends to be misdiagnosed. Surgical resection is a feasible treatment for patients with isolated nodules, especially those with acute obstructive hydrocephalus.
原发性脑室淋巴瘤（PVL）是一种极为罕见且常被误诊的疾病。既往研究主要为病例报告，关于PVL诊断和治疗的文献有限。因此，本研究旨在评估PVL患者的特征。
对经病理确诊的PVL患者的数据进行评估。回顾性分析流行病学数据、影像学表现、手术情况、病理结果及预后。还对相关文献进行了系统综述。
共纳入8例PVL患者。主要症状为颅内压升高。影像学上，5例为单发病变，3例为多发病变；磁共振成像的典型表现包括T1加权像和T2加权像上的低信号、相邻脑水肿以及T1加权像增强扫描上的均匀强化。术前，6例被误诊，2例未明确诊断。所有患者均行开颅手术，4例实现了全切。4例患者手术切除后脑积水得到缓解。病理显示所有患者均为弥漫性大B细胞淋巴瘤。仅1例患者出现严重并发症。共有3例患者接受了辅助治疗，而5例患者拒绝任何辅助治疗。随访时，患者的中位生存时间为15个月。
原发性脑室淋巴瘤主要表现为颅内压升高症状，有多种影像学诊断特征，但仍易被误诊。手术切除对于孤立结节患者，尤其是急性梗阻性脑积水患者是一种可行的治疗方法。
