Secondary CNS Lymphoma

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-       Synchronous CNS and systemic lymphoma at initial presentation (treatment-naïve; TN-SCNSL)
-        CNS relapse without recurrent systemic lymphoma (relapsed isolated CNS lymphoma; RI-SCNSL)
-        Relapsed concomitant systemic and CNS disease following treatment for systemic lymphoma (RC-SCNSL)

 Generally hybrid disease

 Investigations
-  MRI Head w gadolinium
-  PET-CT
-  Testicular US (blood testes barrier influences treatment)
-  Opthalmoscopy/fundoscopy +/- Vitreal biopsy +/- subretinal aspirate – could need RT
-  Lymph node Biopsy 
NB: Worthwhile to remember patient hx re relapses
-  ?Stereotactic Brain Biopsy w/ Intraoperative rapid cytology and rv of frozen sections......NB: Steroids pre-biopsy may yield non-diagnostic results (1/3 if 7 days steroids)
- Correlate with imaging and timescale
-   LP
o   Good for leptomeningeal (15%) which can be missed on MRI
o   CSF protein levels are prognostic
o   Flow cytometry 
o   Cytospin
o   PCR for IGHV rearrangement: sens.

Trial: MARIETTA study, or also known as the IE LSG 42
-        Single arm prospective trial, 75 patients
-        +/- Steroid pre-phase –>MATRIX + RICE alternating induction x3–>CR/PR ->Carmustine-Thiotepa AutoSCT
-        Pre-morbid performance status <=3
-        2-year overall survival for all of those patients included in the trial just under 50%
-        NB: cytaribin omissions if poor performance status
-        RICE (Ritux isophosphamide, carboplatin and etoposide)…NB, peripheral neuropathy and neurotoxicitiy
-        TN-SCNSL best 70% 2 year PFS
-        RI-SCNSL 40% 2 year PFS... can also be given just MATRIX
-        RC-SCNSL 14% 2 year PFS
-        NB if frail elderly, change MATRIX to MARTA

Response assessment :
-        TN-SCNSL and RC-SCNSL 
o   Brain MRI +/- Spine every 2 cycles
o   PET scan every 2-3 cycles
o   PET and MRI pre-auto, determine least partial response
o   End of treatment PET (6-8 weeks post) and MRI

-        RI-SCNSL : MRI brain +/- spine every 2 cycles…PET only if suspicion of progression elsewhere

Relapse post MARIETTA :

-BTKi ?compassionate access vs Trial
-ZUMA7 trial: CAR-T (anti CD19) NB : ICANS/CRS….Approved for DLBCL 12 relapse within 12 months and primary refractory disease that hasn’t responded
-        PALLIATIVE CARE

 NB Immuno-privileged sites :

-        Primary Intraocular Lymphoma :
o   Stage w PET, MRI head, US Testes
o   MATRIX vs MARTA vs PREMAINE as frailty allows (like 1’ CNS) –> AutoSCT
o   +/- Occular RT
o   Frail++ +-> Intravitreal MTX

-        Primary Testicular Lymphoma
o   If 1 testicle involved 1/3 of patients have the other involved too
o   US Testes –> Orchidectomy + histopathology…if lymphoma ->imaging and investigations as above
o   LP with above investigations as 1/3 have CNS involvement
o   ?skin lesions sometimes in testicular lymphoma
o   RCHOP vs RPolaChP + CNS prophylaxis w MTX
o   Radiotherapy (30gy) to contralateral testes to reduce contralateral Relapse risk and/or  BL orchidectomy- fertility discussion
o   System

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