Skip to main content
SpringerLink
Log in

Primary Central Nervous System Lymphoma: Systemic Relapse

  • Chapter
  • First Online:
Tumors of the Central Nervous System, Volume 9

Part of the book series: Tumors of the Central Nervous System ((TCNS,volume 9))

  • 1015 Accesses

Abstract

Primary CNS lymphoma (PCNSL) is characterized by the presence of lymphoma cells limited to the CNS, with possible involvement of the eyes or the cerebrospinal fluid. A majority of PCNSL are diffuse large B-cell lymphomas with an aggressive course. Retrospective series show that about 4% of patients (range, 0–24%) may experience a systemic relapse, mainly with unusual extra-nodal involvement. Systemic relapse can occur very early after initial diagnosis of PCNSL, raising the question of the existence of a systemic lymphoma going undetected at the time of conventional PCNSL staging. Late relapse can occur more than 5 years after the diagnosis, suggesting the development of a second lymphoma or the emergence of a systemic lymphoma arising from circulating clones. Globally, the prognosis is poor but seems slightly more favorable than for patients with intra-cerebral relapse. The present chapter reviews the biological implications of systemic relapse in the pathogenesis of PCNSL.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • Abrey LE, Yahalom J, DeAngelis LM (2000) Treatment for primary CNS lymphoma: the next step. J Clin Oncol 18:3144–3150

    PubMed  CAS  Google Scholar 

  • Abrey LE, Batchelor TT, Ferreri AJ, Gospodarowicz M, Pulczynski EJ, Zucca E, Smith JR, Korfel A, Soussain C, DeAngelis LM, Neuwelt EA, O’Neill BP, Thiel E, Shenkier T, Graus F, van den Bent M, Seymour JF, Poortmans P, Armitage JO, Cavalli F (2005) Report of an international workshop to standardize baseline evaluation and response criteria for primary CNS lymphoma. J Clin Oncol 23:5034–5043

    Article  PubMed  Google Scholar 

  • Angelov L, Doolittle ND, Kraemer DF, Siegal T, Barnett GH, Peereboom DM, Stevens G, McGregor J, Jahnke K, Lacy CA, Hedrick NA, Shalom E, Ference S, Bell S, Sorenson L, Tyson RM, Haluska M, Neuwelt EA (2009) Blood–brain barrier disruption and intra-arterial methotrexate-based therapy for newly diagnosed ­primary CNS lymphoma: a multi-institutional experience. J Clin Oncol 27:3503–3509

    Article  PubMed  CAS  Google Scholar 

  • Blay JY, Conroy T, Chevreau C, Thyss A, Quesnel N, Eghbali H, Bouabdallah R, Coiffier B, Wagner JP, Le Mevel A, Dramais-Marcel D, Baumelou E, Chauvin F, Biron P (1998) High-dose methotrexate for the treatment of primary cerebral lymphomas: analysis of survival and late neurologic toxicity in a retrospective series. J Clin Oncol 16:864–871

    PubMed  CAS  Google Scholar 

  • Chuang SS, Liu H, Huang Y, Chio CC, Lin LC (2009) Primary cerebral diffuse large B-cell lymphoma relapsed solely in the skin with the same clonal origin. Appl Immunohistochem Mol Morphol 17:77–81

    Article  PubMed  Google Scholar 

  • De Jong D, Glas AM, Boerrigter L, Hermus MC, Dalesio O, Willemse E, Nederlof PM, Kersten MJ (2003) Very late relapse in diffuse large B-cell lymphoma represents clonally related disease and is marked by germinal center cell features. Blood 102:324–327

    Article  PubMed  Google Scholar 

  • DeAngelis LM, Seiferheld W, Schold SC, Fisher B, Schultz CJ (2002) Combination chemotherapy and radio­therapy for primary central nervous system lymphoma: Radiation Therapy Oncology Group Study 93–10. J Clin Oncol 20:4643–4648

    Article  PubMed  Google Scholar 

  • Ferreri AJ, Reni M, Zoldan MC, Terreni MR, Villa E (1996) Importance of complete staging in non-Hodgkin’s lymphoma presenting as a cerebral mass lesion. Cancer 77:827–833

    Article  PubMed  CAS  Google Scholar 

  • Ferreri AJ, Dell’Oro S, Foppoli M, Bernardi M, Brandes AA, Tosoni A, Montanari M, Balzarotti M, Spina M, Ilariucci F, Zaja F, Stelitano C, Bobbio F, Corazzelli G, Baldini L, Ponzoni M, Picozzi P, Caligaris CF, Reni M (2006) MATILDE regimen followed by radiotherapy is an active strategy against primary CNS lymphomas. Neurology 66:1435–1438

    Article  PubMed  CAS  Google Scholar 

  • Ferreri AJ, Reni M, Foppoli M, Martelli M, Pangalis GA, Frezzato M, Cabras MG, Fabbri A, Corazzelli G, Ilariucci F, Rossi G, Soffietti R, Stelitano C, Vallisa D, Zaja F, Zoppegno L, Aondio GM, Avvisati G, Balzarotti M, Brandes AA, Fajardo J, Gomez H, Guarini A, Pinotti G, Rigacci L, Uhlmann C, Picozzi P, Vezzulli P, Ponzoni M, Zucca E, Caligaris-Cappio F, Cavalli F (2009) High-dose cytarabine plus high-dose methotrexate versus high-dose methotrexate alone in patients with primary CNS lymphoma: a randomised phase 2 trial. Lancet 374:1512–1520

    Article  PubMed  CAS  Google Scholar 

  • Gavrilovic IT, Hormigo A, Yahalom J, DeAngelis LM, Abrey LE (2006) Long-term follow-up of high-dose methotrexate-based therapy with and without whole brain irradiation for newly diagnosed primary CNS lymphoma. J Clin Oncol 24:4570–4574

    Article  PubMed  CAS  Google Scholar 

  • Gerstner ER, Carson KA, Grossman SA, Batchelor TT (2008) Long-term outcome in PCNSL patients treated with high-dose methotrexate and deferred radiation. Neurology 70:401–402

    Article  PubMed  Google Scholar 

  • Ghesquieres H, Ferlay C, Sebban C, Perol D, Bosly A, Casasnovas O, Reman O, Coiffier B, Tilly H, Morel P, Van den Neste E, Colin P, Haioun C, Biron P, Blay JY (2010) Long-term follow-up of an age-adapted C5R protocol followed by radiotherapy in 99 newly diagnosed primary CNS lymphomas: a prospective multicentric phase II study of the Groupe d’Etude des Lymphomes de l’Adulte (GELA). Ann Oncol 21:842–850

    Article  PubMed  CAS  Google Scholar 

  • Gisselbrecht C, Glass B, Mounier N, Singh GD, Linch DC, Trneny M, Bosly A, Ketterer N, Shpilberg O, Hagberg H, Ma D, Briere J, Moskowitz CH, Schmitz N (2010) Salvage regimens with autologous transplantation for relapsed large B-cell lymphoma in the rituximab era. J Clin Oncol 28:4184–4190

    Article  PubMed  Google Scholar 

  • Herrlinger U, Kuker W, Uhl M, Blaicher HP, Karnath HO, Kanz L, Bamberg M, Weller M (2005) NOA-03 trial of high-dose methotrexate in primary central nervous system lymphoma: final report. Ann Neurol 57:843–847

    Article  PubMed  CAS  Google Scholar 

  • Jahnke K, Hummel M, Korfel A, Burmeister T, Kiewe P, Klasen HA, Muller HH, Stein H, Thiel E (2006a) Detection of subclinical systemic disease in primary CNS lymphoma by polymerase chain reaction of the rearranged immunoglobulin heavy-chain genes. J Clin Oncol 24:4754–4757

    Article  PubMed  CAS  Google Scholar 

  • Jahnke K, Thiel E, Martus P, Herrlinger U, Weller M, Fischer L, Korfel A (2006b) Relapse of primary central nervous system lymphoma: clinical features, outcome and prognostic factors. J Neurooncol 80:159–165

    Article  PubMed  Google Scholar 

  • McAllister LD, Doolittle ND, Guastadisegni PE, Kraemer DF, Lacy CA, Crossen JR, Neuwelt EA (2000) Cogni­tive outcomes and long-term follow-up results after enhanced chemotherapy delivery for primary central nervous system lymphoma. Neurosurgery 46:51–60

    Article  PubMed  CAS  Google Scholar 

  • McCann KJ, Ashton-Key M, Smith K, Stevenson FK, Ottensmeier CH (2009) Primary CNS lymphoma: tumor-related clones exist in the blood and bone marrow with evidence for separate development. Blood 113:4677–4680

    Article  PubMed  CAS  Google Scholar 

  • Mohile NA, DeAngelis LM, Abrey LE (2008) The utility of body FDG PET in staging primary central nervous system lymphoma. Neuro Oncol 10:223–228

    Article  PubMed  Google Scholar 

  • Nayak L, Hedvat C, Rosenblum MK, Abrey LE, DeAngelis LM (2011) Late relapse in primary central nervous system lymphoma: clonal persistence. Neuro Oncol 13:525–529

    Article  PubMed  Google Scholar 

  • O’Brien P, Roos D, Pratt G, Liew K, Barton M, Poulsen M, Olver I, Trotter G (2000) Phase II multicenter study of brief single-agent methotrexate followed by irradiation in primary CNS lymphoma. J Clin Oncol 18:519–526

    PubMed  Google Scholar 

  • Omuro AM, DeAngelis LM, Yahalom J, Abrey LE (2005) Chemoradiotherapy for primary CNS lymphoma: an intent-to-treat analysis with complete follow-up. Neurology 64:69–74

    Article  PubMed  CAS  Google Scholar 

  • Omuro AM, Taillandier L, Chinot O, Carnin C, Barrie M, Hoang-Xuan K (2007) Temozolomide and methotrexate for primary central nervous system lymphoma in the elderly. J Neurooncol 85:207–211

    Article  PubMed  CAS  Google Scholar 

  • O’Neill BP, Dinapoli RP, Kurtin PJ, Habermann TM (1995) Occult systemic non-Hodgkin’s lymphoma (NHL) in patients initially diagnosed as primary central nervous system lymphoma (PCNSL): how much staging is enough? J Neurooncol 25:67–71

    Article  PubMed  Google Scholar 

  • Pels H, Schmidt-Wolf IG, Glasmacher A, Schulz H, Engert A, Diehl V, Zellner A, Schackert G, Reichmann H, Kroschinsky F, Vogt-Schaden M, Egerer G, Bode U, Schaller C, Deckert M, Fimmers R, Helmstaedter C, Atasoy A, Klockgether T, Schlegel U (2003) Primary central nervous system lymphoma: results of a pilot and phase II study of systemic and intraventricular chemotherapy with deferred radiotherapy. J Clin Oncol 21:4489–4495

    Article  PubMed  CAS  Google Scholar 

  • Provencher S, Ferlay C, Alaoui-Slimani K, Devidas A, Lepretre S, de Prijck B, Sebban C, de la Fouchardiere A, Chassagne-Clement C, Ketterer N, Thyss A, Delannoy A, Tilly H, Biron P, Blay JY, Ghesquieres H (2011) Clinical characteristics and outcome of isolated extracerebral relapses of primary central nervous system lymphoma: a case series. Hematol Oncol 29:10–16

    Article  PubMed  Google Scholar 

  • Thiel E, Korfel A, Martus P, Kanz L, Griesinger F, Rauch M, Roth A, Hertenstein B, von Toll T, Hundsberger T, Mergenthaler HG, Leithauser M, Birnbaum T, Fischer L, Jahnke K, Herrlinger U, Plasswilm L, Nagele T, Pietsch T, Bamberg M, Weller M (2010) High-dose methotrexate with or without whole brain radiotherapy for primary CNS lymphoma (G-PCNSL-SG-1): a phase 3, randomised, non-inferiority trial. Lancet Oncol 11:1036–1047

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Medical Oncology, Centre Léon Bérard, Université de Lyon, 128 rue Laennec, 69008, Lyon, France

    Hervé Ghesquières

Authors
  1. Hervé Ghesquières
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Hervé Ghesquières .

Editor information

Editors and Affiliations

  1. , Kean University, Room 213, Library building, Morris Avenue 1000, Union, 07083, New Jersey, USA

    M.A. Hayat

Rights and permissions

Reprints and permissions

Copyright information

© 2012 Springer Science+Business Media Dordrecht

About this chapter

Cite this chapter

Ghesquières, H. (2012). Primary Central Nervous System Lymphoma: Systemic Relapse. In: Hayat, M. (eds) Tumors of the Central Nervous System, Volume 9. Tumors of the Central Nervous System, vol 9. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-5488-1_2

Download citation

  • DOI: https://doi.org/10.1007/978-94-007-5488-1_2

  • Published:

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-007-5487-4

  • Online ISBN: 978-94-007-5488-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation