Listen "Primary CNS Lymphoma"
Episode Synopsis
FeedbackCNS Lymphomas1% of all NHL3% of all Brain tumoursMost common subtype (90%) is DLBCL Clinical division:1. 1* CNS lymphoma, 2. 2* CNS lymphoma- TN-SCNSL- RI-SCNSL- RC-SCNSL3. Immune deficiency assoc- HIV; better prog. Presentation: - SOL Sx - Raised ICP: morning headaches w N+V- Neuropsych, Behavioural, Memory, Language- Focal motor + Stroke Sx- Seizures- Visual Sx and uveitis Investigations:- FBC + Blood film (exclude 2* CNS lymphoma and BM), GFR, U&Es- LDH (prog.)- Virology (Hep+HIV)- IGs, SPEp (paraprotein)- Stereotactic Brain Bx w/ IO rapid cytology and rv of frozen sectionsNB: Steroids pre-biopsy ?non-diagnostic results - LP:.Leptomeningeal*.CSF protein- prognostic.Flow.Cytospin.PCR for IGHV r.- CT Head- MRI H (w gadolinium) +/- spine Staging:-R/O systemic lymphoma-PET/CT-US Testes-Opthalmoscopy/fundoscopy +/- Vitreal biopsy +/- subretinal aspirate-?BMBxPre-treatment:-Baseline neuropsych + cognitive ax-Premorbid performance status: ECOG, Echo, GFR, PMHxDx w/o Bx-MRI-Clinical features-Clonal B cells in CSF/Vitreous fluid and/or PCR IGHV rearrangementTreatment:Induction main: - MATRIX- younger <70- MARTA- older >65Consolidation: - Whole brain RT- BCNU Thiotepa AutoSCT- gold standard if fit...Within 6-8 weeks of the 1st day of final induction: consider for all patients with non-progressive disease (EOT MRI)Trials: IELSG32 study (Leukemia, 2022)- induction + consolidation choices for < 70Induction: 3 arms, MTX + Cyt main- MATRIX- MTX +Cyt + Thiotepa + Ritux -> AutoSCT…..best choice (4 cycles)...7yr 70% survivalConsolidation: efficacy equal AutoSCT and WB-RT, favoured AutoSCT for Sx....MATRIX regimen available on NSSG:- Dose ++ to cross BBB- Folinic Acid rescue*- IVF till MTX levels <0.1 umol/L (1st lvl 48hrs after MTX)- EF >45%- GFR >50NB: stop co-trimoxazole, penicillins, aspirin, NSAIDs, PPIs (inhibit MTX clearance)- MTX build up in 3rd spaces- Stem cell harvest post #2- Treatment related mortality 4-7% mostly in #1- Dose reduce Cytaribin (2/3instead of 4 cycles) if pre-morbid, 25-50% totalMARTA study (Blood, Nov 22): fit for autosct and >65- 2x MTX, cytarabin and rituximab ->AutoSCTPRIMAIN study(2017): not fit for autosct >= 651. 4x MTX, Ritux + PO procarbazine2. 6mo of PO procarbazine as maintenance?WB-RT for residual disease- Palliative if unfit and older:DexTemozolomideWB-RT?IT Chemo in leptomeningealIELSG43 study… favoured AutoSCT PFS and OS to de-escalation consol. Follow Up:- Response Ax with contrast enhanced MRI scan: 1-2mo after consol.- Rpt MRI every 3-4mo for 2 years ++- - CR: MRI NAD, normal eye, clear CSF- Stable: <50% decrease, <25% increase- PR: 50% tumor reduction ?persistent CSF- 'Basics to Brilliance: Haematology Podcast' has been accredited for CPD credit by the Royal College of Pathologists UK. Medical professionals and clinical scientists holding career-grade positions, who are registered with any of the Royal Colleges for CPD, will be eligible to earn 1 credit for every hour of learning. Email: [email protected] Insta: BasicstoBrilliance X: @basics_2_brill Send us your feedback!
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