Lateral intraventricular primary central nervous system lymphoma (LIPCNSL): a review

Background: Lateral intraventricular primary central nervous system lymphoma (LIPCNSL) is an extremely rare intraventricular tumor with high malignancy and has never been systematically described. Aim: To analyze the clinical characteristics and therapeutic strategy of LIPCNSL. Design: Single-center retrospective study. Methods: The clinical manifestation, imaging, treatment and outcomes of 13 patients with LIPCNSL who underwent craniotomy in West China Hospital between December 2008 and April 2018 were retrospectively analyzed. Results: Eleven male and two female patients were enrolled. The mean age was 49.7 years (14-65 years). The frequent manifestations include symptoms of raised intracranial pressure and limb weakness. The mean duration was 1.8 months (1 week to 1 year). The average maximal diameter of tumors was 4.1cm (1.8-6.1 cm). Gross total resection was achieved in 84.6% of patients. Symptoms improved in 69.2% of patients but developed in 30.8% of patients after surgery. The median recurrence-free survival (RFS) and overall survival (OS) were 2.0 months (1-86 months) and 3.0 months (1-124 months). High-dose methotrexate or/and radiotherapy significantly prolonged the RFS and OS (P<0.05). Eight patients (72.7%) experienced relapse and progression. Salvage treatment significantly prolonged survival after relapse (P<0.05). Conclusions: LIPCNSL should be considered as a differential diagnosis of intraventricular tumors. High-dose methotrexate-based chemotherapy with or without radiotherapy should be the first-line treatment, and surgery is only for biopsy and improving symptoms. Long-term intensive follow-up is necessary and active salvage treatment should be performed after relapse.