Official websites use. Share sensitive information only on official, secure websites. Introduction A. Presentation varies depending on the involved regions of the CNS. Standard of care includes methotrexate-based polychemotherapy followed by age-tailored thiotepa-based conditioned autologous stem cell transplantation HDC-ASCT , and in patients unsuitable for HDC-ASCT, consolidation with whole-brain irradiation or single-drug maintenance.
Personalized treatment, primary radiotherapy and only supportive care should be considered in unift, frail patients. Relapse rates are higher in older patients, although prognosis of relapsed patients is poor independent of age. Further research is needed to identify diagnostic biomarkers, more efficacious and less neurotoxic treatments, strategies to improve penetration of drugs into the CNS, and immunotherapies and adoptive cell therapies. Most cases of relapsed disease occur in these organs, although rare cases can relapse outside the CNS.
The brain parenchyma is the most common location 1. This distinction is clearly stated in recent interventional prospective trials, whereas some former cumulative case series or retrospective studies addressing molecular, radiologic, and clinical findings as well as analyzing selected subgroups of patients often included different lymphoma categories arising in or disseminating to the CNS. Conclusions from these retrospective studies should be taken into account with caution considering that PCNSL and other CNS lymphoma categories display remarkably different biological, molecular and clinical findings.
Nevertheless, the molecular and genetic profiles of PCNSL are poorly understood compared with those of nodal DLBCL, mostly due to its rarity and paucity of available biological samples for molecular and biological analyses. B cells are only very rarely found in CNS parenchyma, and PCNSL occurs in organs with structural, biological, and immunological characteristics that strongly influence tumor behavior and both treatment choice and efficacy 5.
Diagnosis of PCNSL has improved in the past few years, leading to faster diagnosis and improved outcomes 7 , 8. Moreover, the molecular profile of PCNSL has been better characterized, which had a positive effect on diagnosis and recognition of potential therapeutic targets. Furthermore, consensus is increasing regarding the optimal treatments of PCNSL, which emphasizes the important role of referral centers. However, despite these advances, PCNSL still has one of the worst prognoses among all non-Hodgkin lymphomas 9 , although it is important to recognize that it is a curable brain tumor.
