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PCNSL - confined to brain, leptomeninges,eyes,spinal cord.~ 2% of primary CNS tumors.median age-65ymc presentation: Focal neurologic deficitsImaging choice : Contrast-enh MRI
Predictors of poor prognosis:age>60 y,ECOG>1, 
LDH,CSF protein ,inv of deep areas
PATHOLOGY (1):90% –DLBCLRest :T-NHL, poorly characterized low-grade NHL, or Burkitt's> 90% PCNS DLBCL-ABC subtype
NEWER INSIGHTS :The B-cell receptor (BCR) signaling axis with its downstream target,NFkB, is affected by mutations in MYD88, CD79B &rarely CARD11.
PATHOLOGY CONT(2)notable risk factor - immunodeficiency HIV infection, iatrogenic immune suppression, and congenital immune deficiency PCNSL may be a consequence of EBV-mediated clonal expansion and malignant transformation of B-lymphocytes
PATHOLOGY CONT (3)Most PCNSL cells demonstrate IgHV genes that have high levels of somatic mutations and show intraclonal heterogeneity pointing towards their derivation from mutated germinal center B cellsHere is a bonus tweet of pathology in PCNSL https://twitter.com/anjuthevirgo/status/1293285265964822528?s=20
MANAGEMENT :Rx: IV HD-MTX at var doses (1–8 g/m2) with other chemotherapeutic agents and/or WBRT
-no consensus on the optimal dose of MTX/on the role of radiation
-Rituximab is incorporated in these regimes.
Here is an awesome yet simplified approach @UpToDate based on age
NOVEL Insights :Modulators of BCR pathway:BTK inhibitor-ibrutinibIMiDs like lenalidomide because of inhibition of NF-κB activity+PI3K/AKT pathway in relapsed refractory cases is promising
Observation of altered PD-L1gene has led to trials of Nivolumab in PCNSL
NOTEWORTHY POINTS:If possible,biopsy shud b done before steroids
Therapy-high dose MTX atleast@3.5g/m2 Despite inadequate data supporting rituximab, excellent tolerability+good results in prospective trials, RTX may be favored IN front line RXDONOT FORGET TESTIS
COMMONLY USED PROTOCOLSWe prefer using the R-MPV protocol in our centre.
kindly add on to this dear seniors @DrGPrakash @IJMPOofficial @bagalbp @DrPMPGI @rahulbhargavadr @BollamRajesh4 @pb10_bmt @blood_academy @prantar @EshaKaul1 @dixit_hemat @HariMenon68 @ReetuJain19 @drkunalsehgal @satya_yadav @DimPositive
