Review ArticlesMarginal Zone LymphomasBertoni, Francesco MD∗,†; Rossi, Davide MD, PhD∗,†; Raderer, Markus MD‡; Zucca, Emanuele MD∗,† Author Information From the ∗Institute of Oncology Research, Faculty of Biomedical Sciences, USI †Oncology Institute of Southern Switzerland (IOSI), Bellinzona, Switzerland ‡Internal Medicine I, Oncology, Medical University of Vienna, Vienna, Austria. Conflicts of Interest and Source of Funding: F.B.: institutional research funds from Acerta, ADC Therapeutics, Bayer AG, Cellestia, CTI Life Sciences, EMD Serono, Helsinn, ImmunoGen, Menarini Ricerche, NEOMED Therapeutics 1, Nordic Nanovector ASA, Oncology Therapeutic Development, and PIQUR Therapeutics AG; consultancy fee from Helsinn and Menarini; expert statements provided to HTG; and travel grants from Amgen, AstraZeneca, Jazz Pharmaceuticals, PIQUR Therapeutics AG. D.R.: honoraria from Abbvie, AstraZeneca, Gilead, Janssen, Loxo, and Verastem and research grants from Abbvie, AstraZeneca, Cellestia, Gilead, and Janssen. M.R.: honoraria from Celgene, EISAI, Novartis, and Ipsen. E.Z.: institutional research funds from Celgene, Roche, and Janssen; advisory board fees from Celgene, Roche, Mei Pharma, AstraZeneca, and Celltrion Healthcare; and travel grants from Abbvie and Gilead; and expert statements provided to Gilead, Bristol-Myers Squibb, and MSD. Reprints: Francesco Bertoni, MD, Institute of Oncology Research, Faculty of Biomedical Sciences, USI, Via Vincenzo Vela 6, CH-6500 Bellinzona, Switzerland. E-mail: [email protected]. The Cancer Journal: 7/8 2020 - Volume 26 - Issue 4 - p 336-347 doi: 10.1097/PPO.0000000000000463 Buy Metrics Abstract There are three different marginal zone lymphoma (MZLs) entities: the extranodal MZL of mucosa- associated lymphoid tissue, the splenic MZL, and the nodal MZL. The 3 MZLs share common lesions (trisomies of chromosomes 3 and 18, deletions at 6q23), and alterations of the nuclear factor κB pathway are frequent events in all of them, but they also differ in the presence of recurrent translocations, mutations affecting the NOTCH pathway, and the transcription factor Kruppel-like factor 2 (KLF2) or the receptor-type protein tyrosine phosphatase delta (PTPRD). This review outlines the most recent and main advances in our understanding of the genetics and biology of MZLs and summarizes the clinical activity of the novel therapeutic approaches targeting the main druggable pathways. The current principles of the standard management of MZL at different anatomic sites are also discussed. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.