Modern concepts in the biology, diagnosis, differential diagnosis and treatment of primary central nervous system lymphoma

• Published in: Leukemia Vol. 25; no. 12; pp. 1797 - 1807
• Main Authors: Deckert, M, Engert, A, Brück, W, Ferreri, A J M, Finke, J, Illerhaus, G, Klapper, W, Korfel, A, Küppers, R, Maarouf, M, Montesinos-Rongen, M, Paulus, W, Schlegel, U, Lassmann, H, Wiestler, O D, Siebert, R, DeAngelis, L M
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 01.12.2011; Nature Publishing Group
• Subjects: 692/420; 692/699/67/1059; 692/699/67/1990/291; 692/700/139; B-cell lymphoma; Biological and medical sciences; Biology; Biopsy; Brain research; Cancer; Cancer Research; Cancer therapies; Care and treatment; Central nervous system; Central Nervous System Neoplasms - diagnosis; Central Nervous System Neoplasms - genetics; Central Nervous System Neoplasms - therapy; Chemotherapy; Critical Care Medicine; Diagnosis; Differential diagnosis; Gene expression; Genetic polymorphisms; Health aspects; Hematologic and hematopoietic diseases; Hematology; Hospitals; Humans; Intensive; Internal Medicine; Leukemia; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphocytes B; Lymphoma; Lymphomas; Medical diagnosis; Medical prognosis; Medical research; Medical sciences; Medicine; Medicine & Public Health; Multiple sclerosis; Nervous system; Neuropathology; Oncology; Pathogenesis; Physiological aspects; Prognosis; Radiation therapy; review; Germany; radiotherapy; pathogenesis; diffuse large B-cell lymphoma; primary CNS lymphoma; chemotherapy; Nervous system diseases; Hematology; Pathogenesis; B cell neoplasm; Malignant hemopathy; Biology; Lymphoid neoplasm; Non Hodgkin lymphoma; Radiotherapy; Differential diagnostic; Cerebral disorder; Primary cerebral lymphoma; Chemotherapy; Treatment; Lymphoproliferative syndrome; Central nervous system disease; Primary; Diffuse large B cell lymphoma; Cancer
• ISSN: 0887-6924; 1476-5551
• DOI: 10.1038/leu.2011.169

Recent studies addressing the molecular characteristics of PCNSL, which is defined as malignant B-cell lymphoma with morphological features of DLBCL, have significantly improved our understanding of the pathogenesis of this lymphoma entity, which is associated with an inferior prognosis as compared with DLBCL outside the CNS. This unfavorable prognosis stimulated intense efforts to improve therapy and induced recent series of clinical studies, which addressed the role of radiotherapy and various chemotherapeutic regimens. This review combines the discussion of diagnosis, differential diagnosis and recent progress in studies addressing the molecular pathogenesis as well as therapeutic options in PCNSL.