Progress in the Diagnosis and Treatment of Primary Central Nervous System Diffuse Large B-Cell Lymphoma

doi:10.13438/j.cnki.jdzk.2021.06.009

Abstract

Abstract: In order to further improve the understanding of primary central nervous system diffuse large B-cell lymphoma (PCNS-DLBCL), PubMed, CNKI and other databases were used to search related literature on the diagnosis and treatment of primary central nervous system lymphoma, and then The pathogenesis, clinical manifestations, diagnostic methods and treatment measures of PCNS-DLBCL are reviewed. PCNS-DLBCL is a subtype of extranodal non-Hodgkin’s lymphoma that can affect any part of the central nervous system. Its pathogenesis includes the presence of Epstein-Barr virus, increased expression of PD-L1 and PD-L2, MYD88 gene mutation and CD79B Gene mutations, etc.; clinical manifestations lack specificity, high degree of malignancy, easy to relapse, and poor prognosis; diagnostic methods include imaging examination, cerebrospinal fluid examination, immunohistochemistry and histopathological examination, pathological examination is the gold standard for diagnosis; PCNS-DLBCL treatment measures include radiotherapy, chemotherapy, autologous stem cell transplantation, and combined radiotherapy and chemotherapy. At present, induction chemotherapy based on high-dose methotrexate is the best treatment plan.

Key words: diffuse large B-cell lymphoma, central nervous system, diagnostic methods, treatment measures, methotrexate

ZHANG Rujiang, ZHANG Yunzhou, GAO Daiquan. Progress in the Diagnosis and Treatment of Primary Central Nervous System Diffuse Large B-Cell Lymphoma[J]. Journal of Jishou University(Natural Sciences Edition), 2021, 42(6): 54-61.

References

[1] OSTROM Q T, GITTLEMAN H, LIAO P, et al. CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2010-2014[J].Neuro-Oncology, 2017, 19(Suppl_5): v1-v88.
[2] SWERDLOW S H, CAMPO E, PILERI S A, et al. The 2016 Revision of the World Health Organization Classification of Lymphoid Neoplasms[J]. Blood, 2016, 127(20): 2375-2390.
[3] ZHANG Yan, ZHOU Daobin. Primary Central Nervous System Lymphoma: Status and Advances in Diagnosis, Molecular Pathogenesis, and Treatment[J]. Chinese Medical Journal, 2020, 133(12): 1462-1469.
[4] VILLANO J L,KOSHY M,SHAIKH H,et al. Age, Gender, and Racial Differences in Incidence and Survival in Primary CNS Lymphoma[J]. British Journal of Cancer,2011,105(9): 1414-1418.
[5] 岳震,冯潇,朱凤仪.原发性中枢神经系统弥漫性大Ｂ细胞淋巴瘤60例分析[J].江苏医药,2018,44(10):1108-1111;1096.
[6] 温延斌,熊海芮,生晓惠,等.原发性中枢神经系统弥漫大B细胞淋巴瘤磁共振及功能成像研究[J].中华临床医师杂志(电子版),2015,9(20):3818-3821.
[7] O'NEILL B P, DECKER P A,TIEU C, et al. The Changing Incidence of Primary Central Nervous System Lymphoma is Driven Primarily by the Changing Incidence in Young and Middle-Aged Men and Differs from Time Trends in Systemic Diffuse Large B-Cell Non-Hodgkin's Lymphoma[J]. American Journal of Hematology, 2013, 88(12): 997-1000.
[8] GIANNINI C, DOGAN A, SALOMO D R. CNS Lymphoma: APractical Diagnostic Approach[J]. Journal of Neuropathology & Experimental Neurology, 2014, 73(6): 478-494.
[9] YANG Xueliang, LIU Yuanbo. Advances in Pathobiology of Primary Central Nervous System Lymphoma[J]. Chinese Medical Journal, 2017, 130(16): 1973-1979.
[10] BHAGAVATHI S, WILSON J D. Primary Central Nervous System Lymphoma[J].Archives of Pathology & Laboratory Medicine, 2008, 132(11): 1830-1834.
[11] CHAPUY B, ROEMER M G M, STEWART C, et al. Targetable Genetic Features of Primary Testicular and Primary Central Nervous System Lymphomas[J]. Blood, 2016, 127(7):869-881.
[12] 梅开勇,林汉良,苏祖兰,等.原发性中枢神经系统淋巴瘤26例临床病理分析术[J].广东医学,2009,30(1):83-85．
[13] PONZONI M, ISSA S, BATCHELOR T T, et al. Beyond High-Dose Methotrexate and Brain Radiotherapy: Novel Targets and Agents for Primary CNS Lymphoma[J]. Annals of Oncology, 2014, 25(2): 316-322.
[14] KADOCH C, TRESELER P, RUBENSTEIN J L. Molecular Pathogenesis of Primary Central Nervous System Lymphoma[J]. Neurosurgical Focus, 2006, 21(5): E1.
[15] SMITH J R, BRAZIEL R M, PAOLETTI S, et al.Expression of B-Cell-Attracting Chemokine 1 (CXCL13) by Malignant Lymphocytes and Vascular Endothelium in Primary Central Nervous System Lymphoma[J]. Blood, 2003, 101(3):815-821.
[16] AHO R, KALIMO H, SALMI M, et al. Binding of Malignant Lymphoid Cells to the White Matter of the Human Central Nervous System: Role of Different CD44 Isoforms, Beta 1, Beta 2 and Beta 7 Integrins, and L-Selectin[J]. Journal of Neuropathology & Experimental Neurology,1997, 56(5): 557-568.
[17] BAIOCCHI R A, KHATRI V P, LINDEMANN M J, et al. Phenotypic and Functional Analysis of Fas (CD95) Expression in Primary Central Nervous System Lymphoma of Patients with Acquired Immunodeficiency Syndrome[J]. Blood, 1997, 90(5): 1737-1746.
[18] GONZALEZ-AGUILAR A, IDBAIH A, BOISSELIER B, et al.Recurrent Mutations of MYD88 and TBL1XR1 in Primary Central Nervous System Lymphomas[J].Clinical Cancer Research, 2012, 18(19): 5203-5211.
[19] NGO V N, YOUNG R M, SCHMITZ R, et al. Oncogenically Active MYD88 Mutations in Human Lymphoma[J]. Nature, 2011, 470(7332): 115-119.
[20] MONTESINOS-RONGEN M, GODLEWSKA E, BRUNN A, et al. Activating L265P Mutations of the MYD88 Gene are Common in Primary Central Nervous System Lymphoma[J]. Acta Neuropathologica, 2011, 122(6): 791-792.
[21] GROMMES C, DEANGELIS L M. Primary CNS Lymphoma[J]. Journal of Clinical Oncology, 2017, 35(21): 2410-2418.
[22] BATAILLE B, DELWAIL V, MENET E, et al. Primary Intracerebral Malignant Lymphoma: Report of 248 Cases[J].Journal of Neurosurgery, 2000, 92(2): 261-266.
[23] CHAN Chichao, RUBENSTEIN J L, COUPLAND S E, et al. Primary Vitreoretinal Lymphoma: A Report from an International Primary Central Nervous System Lymphoma Collaborative Group Symposium[J]. The Oncologist, 2011, 16(11): 1589-1599.
[24] GROMMES C, RUBENSTEIN J L, DEANGELIS L M, et al. Comprehensive Approach to Diagnosis and Treatment of Newly Diagnosed Primary CNS Lymphoma[J]. Neuro-Oncology, 2019, 21(3): 296-305.
[25] 朱铁楠,王书杰,张薇,等.37例原发中枢神经系统淋巴瘤患者的临床特征及预后分析[J].中华血液学杂志,2015,36(10):849-852.
[26] ABREY L E, BATCHELOR T T, FERRERI A J M, et al. Report of an International Workshop to Standardize Baseline Evaluation and Response Criteria for Primary CNS Lymphoma[J]. Journal of Clinical Oncology, 2005, 23(22): 5034-5043.
[27] 王烨,马晓梅,岳娜,等.原发性中枢神经系统弥漫性大B细胞淋巴瘤30例临床病理特征及生存预后分析[J].诊断病理学杂志,2016,23(10):736-741.
[28] 耿承军,陈君坤,卢光明,等.原发性中枢神经系统淋巴瘤的CT、MRI表现与病理对照研究[J].中华放射学杂志,2003,37(3):246-250.
[29] ZOU Yaru, TONG Jianjing, LENG Haiyan, et al. Diagnostic Value of Using 18F-FDG PET and PET/CT in Immunocompetent Patients with Primary Central Nervous System Lymphoma: A Systematic Review and Meta-Analysis[J]. Oncotarget, 2017, 8(25): 41518-41528.
[30] CHUKWUEKE U N, NAYAK L. Central Nervous System Lymphoma[J]. Hematology-Oncology Clinics of North America, 2019, 33(4): 597-611.
[31] ROY S, JOSEPHSON S A, FRIDLYAND J, et al. Protein Biomarker Identification in the CSF of Patients with CNS Lymphoma[J]. Journal of Clinical Oncology, 2008, 26(1): 96-105.
[32] SONG Yang,ZHANG Wei,ZHANG Li, et al. Cerebrospinal Fluid IL-10 and IL-10/IL-6 as Accurate Diagnostic Biomarkers for Primary Central Nervous System Large B-Cell Lymphoma[J]. Scientific Reports, 2016, 6(1): 38671.
[33] PENTSOVA E I, SHAH R H, TANG Jiabin, et al. Evaluating Cancer of the Central Nervous System Through Next-Generation Sequencing of Cerebrospinal Fluid[J].Journal of Clinical Oncology, 2016, 34(20): 2404-2415.
[34] GROMMES C, PASTORE A, PALASKAS N, et al. Ibrutinib Unmasks Critical Role of Bruton Tyrosine Kinase in Primary CNS Lymphoma[J].Cancer Discovery, 2017, 7(9): 1018-1029.
[35] CAMILLERI-BROT S, CRINIRE E, BROT P, et al.A Uniform Activated B-Cell-Like Immunophenotype Might Explain the Poor Prognosis of Primary Central Nervous System Lymphomas: Analysis of 83 Cases[J]. Blood, 2006, 107(1): 190-196.
[36] KIM S, NAM S J, KWON D, et al. MYC and BCL2 Overexpression is Associated with a Higher Class of Memorial Sloan-Kettering Cancer Center Prognostic Model and Poor Clinical Outcome in Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System[J].BMC Cancer, 2016, 16(1): 363.
[37] RUBENSTEIN J L, HSI E D, JOHNSON J L, et al. Intensive Chemotherapy and Immunotherapy in Patients with Newly Diagnosed Primary CNS Lymphoma: CALGB 50202 (Alliance 50202)[J]. Journal of Clinical Oncology, 2013, 31(25):3061-3068.
[38] MILLER D C, HOCHBERG F H, HARRIS N L, et al. Pathology with Clinical Correlations of Primary Central Nervous System Non-Hodgkin's Lymphoma. The Massachusetts General Hospital Experience 1958-1989[J].Cancer, 1994, 74(4): 1383-1397.
[39] HOCHBERG F H, MILLER D C. Primary Central Nervous System Lymphoma[J]. Journal of Neurosurgery: Pediatrics, 1988, 68(6): 835-853.
[40] MENDEZ J S, OSTROM Q T, GITTLEMAN H, et al. The Elderly Left Behind-Changes in Survival Trends of Primary Central Nervous System Lymphoma over the Past 4 Decades[J].Neuro-Oncology, 2018, 20(5): 687-694.
[41] CADY FM, O'NEILL B P, LAW M E,et al. Del(6)(q22) and BCL6 Rearrangements in Primary CNS Lymphoma are Indicators of an Aggressive Clinical Course[J]. Journal of Clinical Oncology, 2008, 26(29): 4814-4819.
[42] BATCHELOR T, CARSON K, O'NEILL A, et al. Treatment of Primary CNS Lymphoma with Methotrexate and Deferred Radiotherapy: A Report of NABTT 96-07[J].Journal of Clinical Oncology, 2003, 21(6): 1044-1049.
[43] HOLDHOFF M, AMBADY P, ABDELAZIZ A, et al. High-Dose Methotrexate with or Without Rituximab in Newly Diagnosed Primary CNS Lymphoma[J]. Neurology, 2014, 83(3): 235-239.
[44] FERRERI A J M, CWYNARSKI K, PULCZYNSKI E, et al. Chemoimmunotherapy with Methotrexate, Cytarabine, Thiotepa, and Rituximab (MATRix Regimen) in Patients with Primary CNS Lymphoma: Results of the First Randomisation of the International Extranodal Lymphoma Study Group-32 (IELSG32) Phase 2 Trial[J].The Lancet Haematology, 2016, 3(5): e217-e227.
[45] MORRIS P G, CORREA D D, YAHALOM J, et al. Rituximab, Methotrexate, Procarbazine, and Vincristine Followed by Consolidation Reduced-Dose Whole-Brain Radiotherapy and Cytarabine in Newly Diagnosed Primary CNS Lymphoma: Final Results and Long-Term Outcome[J].Journal of Clinical Oncology, 2013, 31(31): 3971-3979.
[46] SHAH G D, YAHALOM J, CORREA D D, et al. Combined Immunochemotherapy with Reduced Whole-Brain Radiotherapy for Newly Diagnosed Primary CNS Lymphoma[J]. Journal of Clinical Oncology, 2007, 25(30): 4730-4735.
[47] RENI M, FERRERI A J M, GUHA-THAKURTA N, et al.Clinical Relevance of Consolidation Radiotherapy and Other Main Therapeutic Issues in Primary Central Nervous System Lymphomas Treated with Upfront High-Dose Methotrexate[J]. International Journal of Radiation Oncology, Biology, Physics, 2001, 51(2): 419-425.
[48] THIEL E, KORFEL A, MARTUS P, et al. High-Dose Methotrexate with or Without Whole Brain Radiotherapy for Primary CNS Lymphoma (G-PCNSL-SG-1): A Phase 3, Randomised, Non-Inferiority Trial[J].The Lancet Oncology, 2010, 11(11): 1036-1047.
[49] ADHIKARI N, BISWAS A, GOGIA A, et al. A Prospective Phase II Trial of Response Adapted Whole Brain Radiotherapy after High Dose Methotrexate Based Chemotherapy in Patients with Newly Diagnosed Primary Central Nervous System Lymphoma-Analysis of Acute Toxicity Profile and Early Clinical Outcome[J]. Journal of Neuro-Oncology, 2018, 139 (1): 153-166.
[50] ILLERHAUS G, KASENDA B, IHORST G, et al. High-Dose Chemotherapy with Autologous Haemopoietic Stem Cell Transplantation for Newly Diagnosed Primary CNS Lymphoma: A Prospective, Single-Arm, Phase 2 Trial[J].The Lancet Haematology, 2016, 3(8): e388-e397.
[51] WANG N, GILL C, BETENSKY R, et al. Relapse Patterns in Primary CNS Diffuse Large B-Cell Lymphoma[J]. Neurology, 2015, 84(14): 147.
[52] LANGNER-LEMERCIER S, HOUILLIER C, SOUSSAIN C, et al.Primary CNS Lymphoma at First Relapse/Progression: Characteristics, Management, and Outcome of 256 Patients from the French LOC Network[J].Neuro-Oncology, 2016, 18(9): 1297-1303.
[53] SOUSSAIN C, SUZAN F, HOANG-XUAN K, et al. Results of Intensive Chemotherapy Followed by Hematopoietic Stem-Cell Rescue in 22 Patients with Refractory or Recurrent Primary CNS Lymphoma or Intraocular Lymphoma[J].Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology, 2001,19(3): 742-749.
[54] SOUSSAIN C, SIERRAD R M, RICARD D, et al. Primary CNS Lymphoma in Immunocompetent Patients[J].The oncologist,2009,14(5): 526-539.
[55] SOUSSAIN C,HOANG-XUAN K, TAILLANDIER L, et al. Intensive Chemotherapy Followed by Hematopoietic Stem-Cell Rescue for Refractory and Recurrent Primary CNS and Intraocular Lymphoma: Société Franaise de Greffe de Molle Osseuse-Thérapie Cellulaire[J].Journal of Clinical Oncology, 2008, 26(15): 2512-2518.
[56] GROMMES C, NAYAK L, TUN H W, et al. Introduction of Novel Agents in the Treatment of Primary CNS Lymphoma[J]. Neuro-Oncology, 2019, 21(3): 306-313.
[57] NAYAK L, IWAMOTO F M, LACASCE A, et al.PD-1 Blockade with Nivolumab in Relapsed/Refractory Primary Central Nervous System and Testicular Lymphoma[J]. Blood, 2017, 129(23): 3071-3073.
[58] TU Sanfang, ZHOU Xuan, GUO Zhenling, et al. CD19 and CD70 Dual-Target Chimeric Antigen Receptor T-Cell Therapy for the Treatment of Relapsed and Refractory Primary Central Nervous System Diffuse Large B-Cell Lymphoma[J].Frontiers in Oncology, 2019(9): 1350.