Primary central nervous system lymphomas: EHA–ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up
Cet article présente les recommandations de l'"European Hematology Association" et de l'"European Society for Medical Oncology" pour le diagnostic, le traitement et le suivi d'un lymphome primitif du système nerveux central
Definition, incidence and epidemiology :
Primary diffuse large B-cell lymphoma (DLBCL) of the central nervous system (CNS), termed primary CNS lymphoma (PCNSL), is an aggressive neoplasm presenting with disease limited to the CNS. PCNSL was recognised as a distinct entity by the 2017 World Health Organization (WHO) Classification of Tumours of Haematopoietic and Lymphoid Tissues.1 In the 2022 edition of the WHO classification,2 this neoplasm is classified in the ‘Large B-cell lymphomas of immune-privileged sites’ group, whereas it is considered a specific entity in the International Consensus Classification of Mature Lymphoid Neoplasms.3 This entity is also recognised by the WHO classification of CNS tumours.4 PCNSL can arise in both immunocompetent individuals and in those who are immunosuppressed (e.g. individuals living with human immunodeficiency virus and patients receiving immunosuppressive therapies following organ transplant). While no clear predisposing factors have been recognised in immunocompetent individuals, the nature, intensity and duration of immune suppression can influence the risk of PCNSL in those who are immunocompromised.
Annals of Oncology , article en libre accès, 2023