• Dawei Chen, Xu Yan, Liuzhe Lu, Kun Xue, and Xuechao Dong.
• Department of Neurosurgery, The First Hospital of Jilin University, Changchun, Jilin, China.
• Medicine (Baltimore). 2023 Nov 17; 102 (46): e35954e35954.

RationaleNon-Hodgkin lymphoma affecting the brain, eyes, and cerebrospinal fluid without systemic spread is known as primary central nervous system lymphoma (PCNSL). While intracerebroventricular PCNSL is commonly found in the lateral ventricles and the third and fourth ventricles, the occurrence of PCNSL originating from the septum pellucidum is extremely rare.Patient ConcernsTwo patients presented with recent memory loss and high cranial pressure.DiagnosesMagnetic resonance imaging revealed a clear enhancing lesion in the septum pellucidum region. Pathological examination confirmed that both cases were primary large B-cell lymphoma GCB (germinal center B-cell-like) subtypes located in an "immune-privileged" area.InterventionsBoth patients underwent total tumor resection, and the procedures were successfully completed without surgical complications.OutcomesOver a 1-year period, treatment included four cycles of high-dose methotrexate combined with temozolomide. During the follow-up period (19-23 months), no recurrence of the lymphoma was observed.LessonsIn cases of PCNSL in the septum pellucidum, it is crucial to consider it as a potential differential diagnosis for intraventricular tumors. Surgical interventions should focus on maximizing tumor resection while ensuring the protection of critical structures like the fornix and peripheral neural components. The role of surgery compared to biopsy, as well as the long-term complications, necessitates extended follow-up. Additionally, an individualized treatment approach, considering factors such as age, Karnofsky performance score, and organ function assessment, can lead to positive outcomes.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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