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

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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

Edité par CCSD ; Oxford University Press (OUP) -

International audience. 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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