Neurosurgical Management of Central Nervous System Lymphoma: Lessons Learnt from a Neuro-Oncology Multidisciplinary Team Approach

Abstract

Central nervous system lymphoma (CNSL) represents one of the most aggressive forms of extranodal lymphoma. The gold standard for CNSL diagnosis remains the stereotactic biopsy, with a limited role for cytoreductive surgery that has not been supported by historical data. Our study aims to provide a comprehensive overview of neurosurgery's role in the diagnosis of systemic relapsed and primary CNSL, with an emphasis on the impact on management and survival. This is a single center retrospective cohort study with data collected between August 2012 and August 2020, including patients referred with a potential diagnosis of CNSL to the local Neuro-oncology Multidisciplinary Team (MDT). The concordance between the MDT outcome and histopathological confirmation was assessed using diagnostic statistics. A Cox regression is used for overall survival (OS) risk factor analysis, and Kaplan-Meier statistics are performed for three prognostic models. The diagnosis of lymphoma is confirmed in all cases of relapsed CNSL, and in all but two patients who underwent neurosurgery. For the relapsed CNSL group, the highest positive predictive value (PPV) is found for an MDT outcome when lymphoma had been considered as single or topmost probable diagnosis. Neuro-oncology MDT has an important role in establishing the diagnosis in CNSL, not only to plan tissue diagnosis but also to stratify the surgical candidates. The MDT outcome based on history and imaging has good predictive value for cases where lymphoma is considered the most probable diagnosis, with the best prediction for cases of relapsed CNSL, questioning the need for invasive tissue diagnosis in the latter group.

Keywords: CNS lymphoma; DLBCL; PCNSL; extranodal lymphoma; lymphoma CNS relapse.

Figure 1

Kaplan–Meier survival analysis for relapsed CNSL for each prognostic score (MSKCC prognostic score, Taipei score, NB prediction score).

Figure 1

Kaplan–Meier survival analysis for relapsed CNSL for each prognostic score (MSKCC prognostic score, Taipei score, NB prediction score).

Figure 2

Kaplan–Meier survival analysis for PCNSL for each prognostic score (MSKCC prognostic score, Taipei score, NB prediction score).

Figure 2

Kaplan–Meier survival analysis for PCNSL for each prognostic score (MSKCC prognostic score, Taipei score, NB prediction score).

Figure 3

Kaplan–Meier survival analysis for the entire CNSL cohort for each prognostic score (MSKCC prognostic score, Taipei score, NB prediction score).

Figure 4

Receiver Operating Characteristic (ROC) Curves for each group (Relapsed CNSL, PCNSL and the entire cohort). ROC curves are followed by a table displaying the ROC analysis results for each prognostic score. Note: AUC, area under the curve; SE, standard error.