Clinical characteristics and prognosis of ventricular primary central nervous system lymphoma: a case series and literature review.

BACKGROUND: Primary central nervous system lymphoma (PCNSL) is a rare intracranial tumor, and even rarer when the lesion involves the ventricles. This article aims to evaluate the clinical features of PCNSL lesions involving the ventricles. METHODS: Gathering data from a single institution, we conducted a retrospective analysis of all patients with pathologically proven PCNSL between August 2004 and August 2021 from Xiangya Hospital databases and searched for previously reported cases in PubMed. The MRI signal characteristics, location, size, and hydrocephalus presence were evaluated on the images. RESULTS: We identified 29 new cases and reviewed 22 previously reported cases. The study included 32 (62.75%) males and 19 (37.25%) females, with a median age of 53 years. Of the 22 cases with preoperative imaging available for review, all lesions appeared as isointense or hyperintense on T1WI. Twenty lesions presented as isointense or hypointense, and two cases presented as hyperintense on T2WI. The median Ki-67 of PCNSL involving the ventricle was 80%. BCL-2 was positive in 10/22 (45.45%) cases, and BCL-6 was positive in 18/28 (64.29%) cases. CONCLUSIONS: We found that age ≥ 60 years old and female gender were significant risk factors for survival outcome (P < 0.05). In terms of imaging presentation and prognosis, PCNSL lesions invading the ventricles were similar to those that were non-invasive. Our findings further suggest that ventricular PCNSL may have more diverse signal types on T2WI. Therefore, we recommend that ventricular lymphoma be treated in the same way as non-ventricular PCNSL.