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. 2016 Sep;18(9):1297-303.
doi: 10.1093/neuonc/now033. Epub 2016 Mar 6.

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  1 , Carole Soussain  1 , Hervé Ghesquières  1 , Olivier Chinot  1 , Luc Taillandier  1 , Pierre Soubeyran  1 , Thierry Lamy  1 , Franck Morschhauser  1 , Alexandra Benouaich-Amiel  1 , Guido Ahle  1 , Marie-Pierre Moles-Moreau  1 , Cécile Moluçon-Chabrot  1 , Pascal Bourquard  1 , Ghandi Damaj  1 , Fabrice Jardin  1 , Delphine Larrieu  1 , Emmanuel Gyan  1 , Remy Gressin  1 , Arnaud Jaccard  1 , Sylvain Choquet  1 , Annie Brion  1 , Olivier Casasnovas  1 , Philippe Colin  1 , Oumedaly Reman  1 , Adrian Tempescul  1 , Jean-Pierre Marolleau  1 , Michel Fabbro  1 , Florian Naudet  1 , Khê Hoang-Xuan  1 , Roch Houot  1
Affiliations

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

Sophie Langner-Lemercier et al. Neuro Oncol. 2016 Sep.

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.

Keywords: primary CNS lymphoma; progression; relapse.

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Figures

Fig. 1.
Fig. 1.
Consort diagram.
Fig. 2.
Fig. 2.
OS2 according to independent prognostic factors. (A) Duration of PFS1. (B) Karnofsky performance status. (C) Salvage vs palliative care. (D) Relapsed vs refractory patients.
Fig. 3.
Fig. 3.
OS2 according to the duration of first remission.
See this image and copyright information in PMC

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