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

Sophie Langner-Lemercier 1 Caroline Houillier 2 Carole Soussain 3 Hervé Ghesquières 4 Olivier Chinot 5 Luc Taillandier 6, 7 Pierre Soubeyran 8 Thierry Lamy 9, 1 Franck Morschhauser 10 Alexandra Benouaich-Amiel Guido Ahle Marie-Pierre Moles-Moreau Cécile Moluçon-Chabrot 11 Pascal Bourquard Ghandi Damaj 12 Fabrice Jardin 13 Delphine Larrieu Emmanuel Gyan 14, 15 Rémy Gressin 16, 17 Arnaud Jaccard 18, 19 Sylvain Choquet 20 Annie Brion 21 Olivier Casasnovas 22 Philippe Colin 23 Oumedaly Reman Adrian Tempescul 24, 25 Jean-Pierre Marolleau 26 Michel Fabbro Florian Naudet 9 Khe Hoang-Xuan 27, 28, 29 Roch Houot 30, 31
Abstract : 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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Contributor : Laurent Jonchère <>
Submitted on : Friday, March 18, 2016 - 10:29:52 AM
Last modification on : Wednesday, September 1, 2021 - 9:06:02 AM

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Sophie Langner-Lemercier, Caroline Houillier, Carole Soussain, Hervé Ghesquières, Olivier Chinot, et al.. Primary CNS lymphoma at first relapse/progression: characteristics, management, and outcome of 256 patients from the French LOC network. Neuro-Oncology, Oxford University Press (OUP), 2016, 18 (9), pp.1297-1303. ⟨10.1093/neuonc/now033⟩. ⟨hal-01290413⟩



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