Primary CNS lymphoma at first relapse/progression: characteristics, management, and outcome of 256 patients from the French LOC network


  • Langner-Lemercier Sophie
  • Houillier Caroline
  • Soussain Carole
  • Ghesquières Hervé
  • Chinot Olivier
  • Taillandier Luc
  • Soubeyran Pierre
  • Lamy Thierry
  • Morschhauser Franck
  • Benouaich-Amiel Alexandra
  • Ahle Guido
  • Moles-Moreau Marie-Pierre
  • Moluçon-Chabrot Cécile
  • Bourquard Pascal
  • Damaj Ghandi
  • Jardin Fabrice
  • Larrieu Delphine
  • Gyan Emmanuel
  • Gressin Rémy
  • Jaccard Arnaud
  • Choquet Sylvain
  • Brion Annie
  • Casasnovas Olivier
  • Colin Philippe
  • Reman Oumedaly
  • Tempescul Adrian
  • Marolleau Jean-Pierre
  • Fabbro Michel
  • Naudet Florian
  • Hoang-Xuan Khe
  • Houot Roch


  • Progression
  • Primary CNS lymphoma
  • Relapse
  • Progression

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Background - Treatment of relapsed/refractory (R/R) primary CNS lymphoma (PCNSL) is poorly defined, because randomized trials and large studies are lacking. The aim of this study was to analyze the characteristics, management, and outcome of R/R PCNSL patients after first-line therapy in a nationwide cohort. Methods - We analyzed R/R PCNSL patients following first-line treatment who had been prospectively registered in the database of the French network for oculocerebral lymphoma (LOC) between 2011 and 2014. Results - Among 563 PCNSL patients treated with first-line therapy, we identified 256 with relapsed (n = 93, 16.5%) or refractory (n = 163, 29.0%) disease. Patients who were asymptomatic at relapse/progression (25.5%), mostly diagnosed on routine follow-up neuroimaging, tended to have a better outcome. Patients who received salvage therapy followed by consolidation (mostly intensive chemotherapy plus autologous hematopoietic stem cell transplantation [ICT + AHSCT]) experienced prolonged survival compared with those who did not receive salvage or consolidation therapy. Independent prognostic factors at first relapse/progression were: KPS ≥ 70 vs KPS < 70), sensitivity to first-line therapy (relapsed vs refractory disease), duration of first remission (progression-free survival [PFS] ≥1 y vs <1 y), and management at relapse/progression (palliative care vs salvage therapy). Patients who relapsed early after first-line therapy (ie, PFS < 1 y) had a poor outcome, comparable to that of refractory patients. Conversely, patients experiencing late relapses (PFS ≥ 1 y) and/or undergoing consolidation with ICT + AHSCT experienced prolonged survival. Conclusions - About a third of PCNSL patients are primary refractory to first line treatment. We identified several independent prognostic factors that can guide the management of R/R PCNSL patients.

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