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

Abstract

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 4.1 cm (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.