Secondary cns lymphoma (BSH 2022)

Intro

Rare event —> limited evidence base —> individualised approach often required. For that reason I have only included broad overview notes here, the BSH good practice paper has lots of detail if needed.

Secondary CNS lymphoma includes three groups:

  • CNS + Systemic disease at first presentation, i.e. treatment naive (TN-SCNSL)

  • Isolated CNS relapse after previous treatment for systemic disease (RI-SCNSL)

  • CNS + Systemic relapse after previous treatment for systemic disease (RC-SCNSL)

Relevant because outcomes vary for the different clinical groups

investigations/work-up

Fitness assessment - e.g. Charlson Comorbidity Index (CCI), G8 screening tool, Cumulative Illness Rating Scale

Biopsy where possible

MRI brain +/- whole spine

CSF examination

PET-CT to assess systemic disease

Testicular ultrasound

Slit-lamp examination for intra-occular involvement

intensive Treatment

Evidence base is largely single arm phase 2 trials

Fit patients, <70 yo: best outcomes are with intensive induction followed by autograft

TRM is high —> Consider pre-phase steroids + adjusting first cycle to patient’s performance status

BSH guideline contains helpful algorithms summarising treatment options

MARIETTA (IELSG42) 2021 is preferred UK intensive treatment protocol

  • 3x MATRix —> 3x R-ICE —> Autograft.

  • All patients 2yr OS = 46%

  • 2yr PFS = 71% TN-SCNSL, 14% RC-SCNSL, 40% RI-SCNSL

TN-SCNSL

Intensive treatment may gain outcomes similar to DLBCL without CNS involvement

RI-SCNSL

Better outcomes than RC-SCNSL, and can be similar to TN-SCNSL

Intensive treatment may gain 2yr PFS of 60-70% (although not see in MARIETTA)

RC-SCNSL

Poor outcomes with intensive treatments, most patients do not make it to autograft

Consider a palliative approach

other treatments

Palliative Care

Best supportive care / Steroids / low dose radiotherapy

Whole brain radiotherapy (WBRT)

36Gy in 20# may achieve durable remission but very high rates of neurotoxicity / early dementia

CAR-T

Small no. of CNS patients in CAR-T studies, e.g. TRANSCEND 2020, show some response

Others

e.g. Lenalidomide & BTK inhibitors cross blood brain barrier but unlincensed.