Table 4 Distinctive features of primary large B-cell lymphomas of immune privileged sites.
Subtypes | Primary large B-cell lymphoma of the CNS |
Primary large B-cell lymphoma of the vitreoretina | |
Primary large B-cell lymphoma of the testis | |
Clinical | Usually in adults over age of 60 years |
Lymphoma tends to “home” to other immune privileged sites: vitreoretina tumour may occur concurrently with or follow CNS tumour; testicular tumour tends to relapse in CNS or contralateral testis | |
Aggressive tumours with generally poor prognosis | |
Morphology | Large B-cell lymphoma |
Immunophenotype | Activated B-cell immunophenotype: Usually CD10-, MUM1+, BCL6+ |
EBV negative | |
Mutational profile | Concomitant MYD88 and CD79B mutations |
Immune evasion: genetic inactivation of MHC class I and II and B2M (β 2-microglobulin) with subsequent loss of protein expression | |
Showing DLBCL genomic signature C5/MCD/MYD88 |
