European Association of Neuro-Oncology (EANO) guidelines for treatment of primary central nervous system lymphoma (PCNSL).

Khê Hoang-Xuan, Martina Deckert, Andrés J M Ferreri, Julia Furtner, Jaime Gallego Perez-Larraya, Roger Henriksson, Andreas F Hottinger, Benjamin Kasenda, Florence Lefranc, Alexander Lossos, Catherine McBain, Matthias Preusser, Patrick Roth, Roberta Rudà, Uwe Schlegel, Riccardo Soffietti, Carole Soussain, Martin J B Taphoorn, Valérie Touitou, Michael Weller, Jacoline E C Bromberg
Author Information
  1. Khê Hoang-Xuan: APHP, Department of Neurology, Groupe Hospitalier Pitié-Salpêtrière; Sorbonne Université; IHU; ICM. Paris, France.
  2. Martina Deckert: Institute of Neuropathology, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany.
  3. Andrés J M Ferreri: Lymphoma Unit, IRCCS San Raffaele Scientific Institute, Milano, Italy.
  4. Julia Furtner: Department of Biomedical and Imaging Image-guided Therapy Medical University of Vienna, Vienna, Austria.
  5. Jaime Gallego Perez-Larraya: Health Research Institute of Navarra (IdiSNA), Program in Solid Tumors, Foundation for the Applied Medical Research, Department of Neurology, Clinica Universidad de Navarra, Pamplona, Navarra, Spain.
  6. Roger Henriksson: Department of Radiation Sciences, Oncology, University of Umeå, S-901 85 Umea, Sweden.
  7. Andreas F Hottinger: Department of Oncology and Clinical Neurosciences, CHUV University Hospital Lausanne and University of Lausanne, LausanneSwitzerland.
  8. Benjamin Kasenda: Department of Hematology/Oncology and Palliative Care, Klinikum Stuttgart, Stuttgart, Germany.
  9. Florence Lefranc: Department of Neurosurgery, Hôpital Erasme, Université Libre de Bruxelles, Belgium.
  10. Alexander Lossos: Head, Leslie and Michael Gaffin Center for Neuro-Oncology; Department of Oncology and Neurology; Hadassah-Hebrew University Medical Center; Jerusalem, Israel.
  11. Catherine McBain: Department of Clinical Oncology, The Christie NHS FT; Manchester; United Kingdom.
  12. Matthias Preusser: Division of Oncology, Department of Medicine I, Medical University of Vienna, Vienna,Austria. ORCID
  13. Patrick Roth: Department of Neurology & Brain Tumor Center, University Hospital and University of Zurich, Zurich, Switzerland.
  14. Roberta Rudà: Department of Neurology, Castelfranco Veneto/Treviso Hospital, Italy.
  15. Uwe Schlegel: Universitätsklinikum Knappschaftskrankenhaus Bochum GmbH, Germany.
  16. Riccardo Soffietti: Division of Neuro-Oncology, Department of Neuroscience, University of Turin, and City of Health and Science University Hospital, Turin, Italy. ORCID
  17. Carole Soussain: Department of Hematology, Institut Curie, Site Saint-Cloud, France and INSERM U932 Institut Curie, PSL Research University, Paris, France.
  18. Martin J B Taphoorn: Department of Neurology, Leiden University Medical Center and Department of Neurology, Haaglanden Medical Center The Hague, The Netherlands.
  19. Valérie Touitou: APHP, Department of Ophtalmology, Groupe Hospitalier Pitié-Salpêtrière; Sorbonne Université. Paris, France.
  20. Michael Weller: Department of Neurology & Brain Tumor Center, University Hospital and University of Zurich, Zurich, Switzerland. ORCID
  21. Jacoline E C Bromberg: Department of Neuro-Oncology, Erasmus MC University Medical Center Cancer Institute, Rotterdam. The Netherlands.

Abstract

The management of primary central nervous system (PCNSL) is one of the most controversial topics in neuro-oncology because of the complexity of the disease and the limited number of controlled studies available. In 2021, given recent advances and the publication of practice-changing randomized trials, the European Association of Neuro-Oncology (EANO) created a multidisciplinary task force to update the previously published evidence-based guidelines for immunocompetent adult patients with PCNSL and added a section on immunosuppressed patients. The guideline provides consensus considerations and recommendations for the treatment of PCNSL, including intraocular manifestations and specific management of the elderly. The main changes from the previous guideline include strengthened evidence for the consolidation with ASCT in first-line treatment, prospectively assessed chemotherapy combinations for both young and elderly patients, clarification of the role of rituximab even though the data remain inconclusive, of the role of new agents, and the incorporation of immunosuppressed patients and primary ocular lymphoma. The guideline should aid the clinicians in everyday practice and decision making and serve as a basis for future research in the field.

Keywords

References

  1. Cancer. 2010 Feb 15;116(4):863-70 [PMID: 20052713]
  2. Cancer. 2004 Jun 15;100(12):2627-36 [PMID: 15197806]
  3. Onco Targets Ther. 2020 Aug 20;13:8323-8335 [PMID: 32903865]
  4. Ann Oncol. 2005 Mar;16(3):445-9 [PMID: 15653703]
  5. J Clin Oncol. 2012 Apr 1;30(10):e119-21 [PMID: 22355047]
  6. Blood Adv. 2021 Oct 26;5(20):4059-4063 [PMID: 34492703]
  7. Ann Oncol. 2012 Jul;23(7):1809-12 [PMID: 22115927]
  8. J Clin Oncol. 2003 Dec 15;21(24):4483-8 [PMID: 14597741]
  9. Lancet. 2009 Oct 31;374(9700):1512-20 [PMID: 19767089]
  10. Am J Transplant. 2013 Jun;13(6):1512-22 [PMID: 23721553]
  11. Lancet Haematol. 2017 Nov;4(11):e510-e523 [PMID: 29054815]
  12. Bone Marrow Transplant. 2017 Aug;52(8):1113-1119 [PMID: 28436974]
  13. Int J Radiat Oncol Biol Phys. 2006 Aug 1;65(5):1429-39 [PMID: 16863926]
  14. Haematologica. 2021 Feb 01;106(2):597-600 [PMID: 32241841]
  15. Cancer. 2007 Dec 1;110(11):2528-34 [PMID: 17932895]
  16. Clin Cancer Res. 2003 Feb;9(2):711-5 [PMID: 12576439]
  17. J Acquir Immune Defic Syndr Hum Retrovirol. 1998 Aug 1;18(4):365-71 [PMID: 9704942]
  18. J Clin Oncol. 2006 Oct 1;24(28):4570-4 [PMID: 17008697]
  19. Br J Haematol. 2010 Oct;151(2):179-84 [PMID: 20738305]
  20. J Clin Oncol. 1998 Mar;16(3):864-71 [PMID: 9508167]
  21. Blood. 1995 Oct 15;86(8):2922-9 [PMID: 7579384]
  22. Cancer. 1974 Oct;34(4):1293-302 [PMID: 4607602]
  23. Neurosurg Rev. 2018 Oct;41(4):1037-1044 [PMID: 29383600]
  24. Int J Radiat Oncol Biol Phys. 1992;23(1):9-17 [PMID: 1572835]
  25. J Clin Oncol. 2019 Apr 1;37(10):823-833 [PMID: 30785830]
  26. Clin Lymphoma Myeloma Leuk. 2019 Mar;19(3):e129-e141 [PMID: 30584023]
  27. Leuk Lymphoma. 2020 Jul;61(7):1766-1768 [PMID: 32204633]
  28. Cancer. 1989 May 15;63(10):1918-21 [PMID: 2702565]
  29. Blood. 2020 May 14;135(20):1811-1815 [PMID: 32125361]
  30. J Neurooncol. 2002 Jun;58(2):175-8 [PMID: 12164690]
  31. J Clin Oncol. 2013 Sep 1;31(25):3061-8 [PMID: 23569323]
  32. Ann Oncol. 2006 Jul;17(7):1141-5 [PMID: 16603598]
  33. Neurology. 2000 Apr 25;54(8):1707-8 [PMID: 10762527]
  34. Cancer. 2008 Sep 1;113(5):1025-31 [PMID: 18618509]
  35. Neurology. 2004 Feb 24;62(4):548-55 [PMID: 14981169]
  36. Cancer Cell. 2017 Jun 12;31(6):833-843.e5 [PMID: 28552327]
  37. Cancer. 2010 Oct 1;116(19):4605-12 [PMID: 20572045]
  38. Leukemia. 2017 Aug;31(8):1822-1825 [PMID: 28450731]
  39. Haematologica. 2012 Nov;97(11):1751-6 [PMID: 22581000]
  40. Neuro Oncol. 2013 Aug;15(8):1068-73 [PMID: 23502429]
  41. Leukemia. 2017 Dec;31(12):2623-2629 [PMID: 28559537]
  42. J Clin Oncol. 2005 Mar 1;23(7):1507-13 [PMID: 15735126]
  43. J Neurooncol. 2019 Aug;144(1):107-115 [PMID: 31190317]
  44. Cancer. 2003 Jan 1;97(1):128-33 [PMID: 12491514]
  45. J Neurooncol. 2014 May;118(1):155-62 [PMID: 24584709]
  46. Am J Transplant. 2018 Feb;18(2):453-461 [PMID: 28805292]
  47. J Neurooncol. 2019 Sep;144(3):553-562 [PMID: 31377920]
  48. Cancer Discov. 2017 Sep;7(9):1018-1029 [PMID: 28619981]
  49. J Neurooncol. 2010 May;97(3):389-92 [PMID: 19841864]
  50. Ann Oncol. 2007 Nov;18(11):1851-5 [PMID: 17804469]
  51. Neurology. 2014 Jul 15;83(3):235-9 [PMID: 24928128]
  52. Leukemia. 2017 Apr;31(4):846-852 [PMID: 27843136]
  53. Neurology. 2021 Sep 28;97(13):628-631 [PMID: 34580183]
  54. Neuro Oncol. 2018 Apr 9;20(5):687-694 [PMID: 29036697]
  55. Clin Lymphoma Myeloma Leuk. 2020 Nov;20(11):e930-e933 [PMID: 32782203]
  56. Neurology. 2014 Apr 15;82(15):1370-3 [PMID: 24634458]
  57. Neurosurgery. 2019 Apr 1;84(4):935-944 [PMID: 29660011]
  58. J Clin Oncol. 2009 Jul 20;27(21):3503-9 [PMID: 19451444]
  59. Am J Hematol. 2013 Dec;88(12):997-1000 [PMID: 23873804]
  60. Retina. 2011 Mar;31(3):435-40 [PMID: 21336066]
  61. Neuro Oncol. 2021 Aug 2;23(8):1315-1326 [PMID: 33560442]
  62. Transl Vis Sci Technol. 2012 Oct 22;1(3):1 [PMID: 24049708]
  63. Neurology. 2002 May 28;58(10):1513-20 [PMID: 12034789]
  64. Arch Neurol. 2005 Oct;62(10):1595-600 [PMID: 16216945]
  65. J Neurooncol. 2016 Feb;126(3):545-50 [PMID: 26563190]
  66. J Neurooncol. 2011 Nov;105(2):409-14 [PMID: 21656329]
  67. AIDS. 2001 Nov 9;15(16):2119-27 [PMID: 11684931]
  68. Neurology. 2013 Jul 2;81(1):84-92 [PMID: 23685932]
  69. Sci Rep. 2021 Jan 22;11(1):2125 [PMID: 33483528]
  70. JAMA Oncol. 2021 Jul 01;7(7):993-1003 [PMID: 33956047]
  71. Br J Ophthalmol. 1989 May;73(5):342-6 [PMID: 2659067]
  72. Am J Hematol. 2021 Jul 1;96(7):823-833 [PMID: 33864703]
  73. Neuro Oncol. 2009 Apr;11(2):211-5 [PMID: 18757775]
  74. Ann Oncol. 2002 Apr;13(4):531-8 [PMID: 12056702]
  75. Blood. 2017 Jun 8;129(23):3071-3073 [PMID: 28356247]
  76. JAMA Ophthalmol. 2015 Feb;133(2):191-7 [PMID: 25412269]
  77. Ann Neurol. 2010 Feb;67(2):182-9 [PMID: 20225195]
  78. Clin Cancer Res. 2004 Sep 1;10(17):5643-6 [PMID: 15355887]
  79. Lancet Oncol. 2018 Aug;19(8):e407-e418 [PMID: 30102235]
  80. J Neurosurg. 2000 Feb;92(2):261-6 [PMID: 10659013]
  81. Nat Rev Clin Oncol. 2021 Jul;18(7):401-417 [PMID: 33654306]
  82. Blood. 2022 Feb 3;139(5):792-796 [PMID: 34871363]
  83. Eur J Neurol. 2004 Sep;11(9):577-81 [PMID: 15379736]
  84. Int J Hematol. 2010 Nov;92(4):617-23 [PMID: 20967518]
  85. Neurosurgery. 2000 Jan;46(1):51-60; discussion 60-1 [PMID: 10626935]
  86. Ann Oncol. 2015 Jul;26(7):1305-13 [PMID: 25701456]
  87. J Gen Virol. 2005 Mar;86(Pt 3):551-559 [PMID: 15722514]
  88. Lancet Oncol. 2019 Feb;20(2):216-228 [PMID: 30630772]
  89. Leuk Lymphoma. 2011 Oct;52(10):1882-90 [PMID: 21663504]
  90. J Clin Oncol. 2013 Nov 1;31(31):3971-9 [PMID: 24101038]
  91. Leukemia. 2022 Jul;36(7):1870-1878 [PMID: 35562406]
  92. Neurology. 2004 Feb 24;62(4):544-7 [PMID: 14981168]
  93. J Clin Oncol. 1996 Feb;14(2):556-64 [PMID: 8636771]
  94. Eye (Lond). 2007 Sep;21(9):1198-201 [PMID: 16732210]
  95. J Neurooncol. 2007 Nov;85(2):207-11 [PMID: 17896079]
  96. Blood. 2003 Jan 15;101(2):466-8 [PMID: 12393404]
  97. Lancet Oncol. 2015 Jul;16(7):e322-32 [PMID: 26149884]
  98. J Cancer Res Clin Oncol. 2017 Sep;143(9):1815-1821 [PMID: 28434043]
  99. Curr Opin Oncol. 2021 Sep 1;33(5):420-431 [PMID: 34292201]
  100. J Neurooncol. 2014 Mar;117(1):161-5 [PMID: 24481997]
  101. J Neurooncol. 2009 Jun;93(2):213-7 [PMID: 19099202]
  102. Am J Clin Pathol. 2004 Feb;121(2):246-53 [PMID: 14983939]
  103. Haematologica. 2013 May;98(5):765-70 [PMID: 23300179]
  104. J Neurooncol. 2012 Jan;106(1):143-6 [PMID: 21739169]
  105. Neuro Oncol. 2012 Oct;14(10):1304-11 [PMID: 22952196]
  106. Int J Radiat Oncol Biol Phys. 2011 May 1;80(1):169-75 [PMID: 20584577]
  107. J Clin Oncol. 2022 Nov 10;40(32):3692-3698 [PMID: 35834762]
  108. J Clin Oncol. 2003 Mar 15;21(6):1044-9 [PMID: 12637469]
  109. Clin Infect Dis. 2006 Feb 15;42(4):e21-5 [PMID: 16421782]
  110. Neuro Oncol. 2021 Jan 30;23(1):122-133 [PMID: 32583848]
  111. J Clin Oncol. 2003 Dec 15;21(24):4489-95 [PMID: 14597744]
  112. Leuk Lymphoma. 2015 Feb;56(2):361-7 [PMID: 24745937]
  113. BMC Neurol. 2021 May 11;21(1):190 [PMID: 33975554]
  114. Int Urol Nephrol. 2013 Aug;45(4):1219-22 [PMID: 22476860]
  115. Br J Ophthalmol. 2008 Mar;92(3):383-8 [PMID: 18303160]
  116. Ann Oncol. 2019 Apr 1;30(4):621-628 [PMID: 30698644]
  117. Neurology. 2017 Aug 22;89(8):796-804 [PMID: 28747447]
  118. Blood Adv. 2018 Jul 10;2(13):1595-1607 [PMID: 29986852]
  119. Hematol Oncol. 2011 Mar;29(1):10-6 [PMID: 21381074]
  120. Eur J Cancer. 2019 Aug;117:121-130 [PMID: 31279304]
  121. Blood. 2018 Nov 22;132(21):2240-2248 [PMID: 30262659]
  122. Hematol Oncol. 2019 Dec;37(5):548-557 [PMID: 31418878]
  123. J Clin Oncol. 2003 Jul 15;21(14):2726-31 [PMID: 12860951]
  124. Neurology. 2011 Mar 8;76(10):929-30 [PMID: 21383331]
  125. Haematologica. 2018 Jul;103(7):e296-e299 [PMID: 29472354]
  126. J Clin Oncol. 2003 Nov 15;21(22):4151-6 [PMID: 14615443]
  127. J Neurooncol. 2011 Aug;104(1):323-30 [PMID: 21170569]
  128. Cancer. 2012 Aug 1;118(15):3743-8 [PMID: 22179954]
  129. J Clin Oncol. 2018 Aug 1;36(22):2326-2347 [PMID: 29782209]
  130. Blood. 2022 Apr 14;139(15):2306-2315 [PMID: 35167655]
  131. Curr HIV Res. 2006 Jul;4(3):375-8 [PMID: 16842088]
  132. J Neurooncol. 2011 Aug;104(1):191-3 [PMID: 21103909]
  133. Neurology. 2002 Nov 26;59(10):1557-62 [PMID: 12451197]
  134. Blood. 2019 Jan 31;133(5):436-445 [PMID: 30567753]
  135. J Clin Oncol. 2001 Feb 1;19(3):742-9 [PMID: 11157026]
  136. Eur J Haematol. 2003 Apr;70(4):219-24 [PMID: 12656744]
  137. Blood. 2020 Nov 5;136(19):2229-2232 [PMID: 32609814]
  138. Onco Targets Ther. 2015 Oct 01;8:2771-3 [PMID: 26491351]
  139. J Clin Oncol. 1992 Apr;10(4):635-43 [PMID: 1548527]
  140. Br J Haematol. 2004 Jul;126(2):202-8 [PMID: 15238140]
  141. Neuro Oncol. 2016 Sep;18(9):1297-303 [PMID: 26951382]
  142. Ann Oncol. 2010 Apr;21(4):842-850 [PMID: 19914958]
  143. J Clin Oncol. 2016 May 20;34(15):1757-63 [PMID: 26976424]
  144. Hematol Oncol. 2013 Sep;31(3):143-50 [PMID: 23161567]
  145. J Neurooncol. 2017 Mar;132(1):189-197 [PMID: 28116650]
  146. Leuk Lymphoma. 2012 May;53(5):862-7 [PMID: 22023529]
  147. Int J Radiat Oncol Biol Phys. 2020 Jul 1;107(3):398-400 [PMID: 32531380]
  148. J Clin Oncol. 2008 May 20;26(15):2512-8 [PMID: 18413641]
  149. Haematologica. 2015 Apr;100(4):534-40 [PMID: 25480497]
  150. Ann Neurol. 2002 Feb;51(2):247-52 [PMID: 11835382]
  151. Neurology. 2007 Sep 11;69(11):1178-82 [PMID: 17846417]
  152. Blood Adv. 2020 Jul 28;4(14):3378-3381 [PMID: 32722778]
  153. Neurology. 2012 Aug 28;79(9):890-6 [PMID: 22895585]
  154. Expert Rev Anticancer Ther. 2013 Nov;13(11):1327-37 [PMID: 24152125]
  155. Front Oncol. 2021 Apr 29;11:649789 [PMID: 33996566]
  156. Neuro Oncol. 2010 Jul;12(7):736-44 [PMID: 20511181]
  157. Int J Hematol. 2013 Sep;98(3):346-54 [PMID: 23868695]
  158. Eur J Cancer. 2004 Jul;40(11):1682-8 [PMID: 15251157]
  159. Lancet Haematol. 2016 May;3(5):e217-27 [PMID: 27132696]
  160. Neurology. 2008 Oct 21;71(17):1355-60 [PMID: 18936428]
  161. Lancet Oncol. 2010 Nov;11(11):1036-47 [PMID: 20970380]
  162. J Neurooncol. 2012 Sep;109(2):285-91 [PMID: 22570142]
  163. Blood Adv. 2020 Aug 11;4(15):3648-3658 [PMID: 32766857]
  164. Neuro Oncol. 2012 Jan;14(1):101-8 [PMID: 22013168]
  165. Ann Hematol. 2019 Apr;98(4):915-922 [PMID: 30535802]
  166. J Clin Oncol. 1998 Mar;16(3):859-63 [PMID: 9508166]
  167. J Neurosurg. 1999 Aug;91(2):221-30 [PMID: 10433310]
  168. Leuk Lymphoma. 2013 Jan;54(1):58-61 [PMID: 22656234]
  169. Oncologist. 2011;16(11):1589-99 [PMID: 22045784]
  170. Int J Radiat Oncol Biol Phys. 1995 Oct 15;33(3):663-73 [PMID: 7558957]
  171. Bone Marrow Transplant. 2022 Jun;57(6):966-974 [PMID: 35422077]
  172. J Neurooncol. 2009 Feb;91(3):299-305 [PMID: 18931887]
  173. Nat Rev Cancer. 2014 Apr;14(4):219-32 [PMID: 24658273]
  174. Curr Opin Oncol. 2011 Nov;23(6):648-53 [PMID: 21946245]
  175. Ann Oncol. 2007 Jul;18(7):1145-51 [PMID: 17284616]
  176. Neuro Oncol. 2017 Jan;19(1):99-108 [PMID: 27576871]
  177. Lancet Haematol. 2015 Jun;2(6):e251-9 [PMID: 26688235]
  178. J Clin Oncol. 2002 Dec 15;20(24):4643-8 [PMID: 12488408]
  179. Neurology. 2020 Dec 8;95(23):e3138-e3144 [PMID: 32989105]
  180. AIDS. 2003 Aug 15;17(12):1787-93 [PMID: 12891064]
  181. Neuro Oncol. 2012 Dec;14(12):1481-4 [PMID: 22984018]
  182. Ann Oncol. 2011 Sep;22(9):2080-2085 [PMID: 21303800]
  183. Ann Oncol. 2009 Feb;20(2):319-25 [PMID: 18953065]
  184. Haematologica. 2008 Jan;93(1):147-8 [PMID: 18166803]

MeSH Term

Adult
Humans
Aged
Combined Modality Therapy
Central Nervous System Neoplasms
Antineoplastic Combined Chemotherapy Protocols
Central Nervous System
Lymphoma

Word Cloud

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