Primary CNS Lymphoma

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CNS Lymphomas

1% of all NHL
3% of all Brain tumours
Most common subtype (90%) is DLBCL

 Clinical division:
1.  1* CNS lymphoma, 
2.  2* CNS lymphoma
- TN-SCNSL
- RI-SCNSL
- RC-SCNSL
3.  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 sections
NB: 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
-?BMBx

Pre-treatment:
-Baseline neuropsych + cognitive ax
-Premorbid performance status: ECOG, Echo, GFR, PMHx

Dx w/o Bx
-MRI
-Clinical features
-Clonal B cells in CSF/Vitreous fluid    and/or   PCR IGHV rearrangement

Treatment:
Induction main:
- MATRIX- younger <70
- MARTA- older >65
Consolidation: 
-  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 < 70
Induction: 3 arms, MTX + Cyt main
-   MATRIX- MTX +Cyt + Thiotepa + Ritux -> AutoSCT…..best choice (4 cycles)...7yr 70% survival
Consolidation: 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 >50
NB: 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% total

MARTA study (Blood, Nov 22): fit for autosct and >65
-   2x MTX, cytarabin and rituximab ->AutoSCT

PRIMAIN study(2017): not fit for autosct >= 65
1.     4x MTX, Ritux + PO procarbazine
2.     6mo of PO procarbazine as maintenance
?WB-RT for residual disease

-   Palliative if unfit and older:
Dex
Temozolomide
WB-RT
?IT Chemo in leptomeningeal

IELSG43 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
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