Primary central nervous system lymphomas: EHA–ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up
Andrés J.M. Ferreri, Gerald Illerhaus, Jeanette K. Doorduijn, Dorothee P. Auer, Jacoline E. C. Bromberg, Teresa Calimeri, Kate Cwynarski, Christopher P. Fox, Khê Hoang‐Xuan, Denis Malaise, Maurilio Ponzoni, Elisabeth Schorb, C. Soussain, Lena Specht, Emanuele Zucca, C. Buske, Mats Jerkeman, M. Dreyling
Abstract
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.