Sophie Langner-Lemercier1, Caroline Houillier1, Carole Soussain1, Hervé Ghesquières1, Olivier Chinot1, Luc Taillandier1, Pierre Soubeyran1, Thierry Lamy1, Franck Morschhauser1, Alexandra Benouaich-Amiel1, Guido Ahle1, Marie-Pierre Moles-Moreau1, Cécile Moluçon-Chabrot1, Pascal Bourquard1, Ghandi Damaj1, Fabrice Jardin1, Delphine Larrieu1, Emmanuel Gyan1, Remy Gressin1, Arnaud Jaccard1, Sylvain Choquet1, Annie Brion1, Olivier Casasnovas1, Philippe Colin1, Oumedaly Reman1, Adrian Tempescul1, Jean-Pierre Marolleau1, Michel Fabbro1, Florian Naudet1, Khê Hoang-Xuan1, Roch Houot1. 1. Department of Neurology, Centre Hospitalier Universitaire de Rennes, Rennes, France (S.L.-L.); Department of Neurology Mazarin, APHP, Sorbonne Universités UPMC Univ Paris VI, IHU, ICM, INCa-LOC Network, Groupe Hospitalier Pitié-Salpétrière, Paris, France (C.H., K.H.-X.); Department of Hematology, Hôpital René Huguenin-Institut Curie, Saint-Cloud, Collège de France, Paris, France (C.S.); Department of Hematology, Centre Léon Bérard, Lyon, France (H.G.); Department of Neurooncology, Centre Hospitalier Universitaire La Timone, Assistance Publique-Hôpitaux de Marseille, INSERM U911, CRO2, Université de la Méditerranée, Marseille, France (O.C.); Department of Neurology, Centre Hospitalier Universitaire de Nancy, Nancy, France (L.T.); Department of Hematology, Institut Bergonié, Bordeaux, France (P.S.); Department of Hematology, Centre Hospitalier Universitaire de Rennes, INSERM U917, Rennes, France (T.L., R.H.); Department of Hematology, Centre Hospitalier Universitaire de Lille, Lille, France (F.M.); Department of Neurology, Centre Hospitalier Universitaire Toulouse-Purpan, Toulouse, France (A.B.-A.); Department of Neurology, Hôpitaux Civils de Colmar, Colmar, France (G.A.); Department of Hematology, Centre Hospitalier Universitaire d'Angers, Angers, France (M.-P.M.-M.); Department of Hematology, Centre Hospitalier Universitaire de Clermont-Ferrand, Clermont-Ferrand, France (C.M.-C.); Department of Hematology and Oncology, Centre Hospitalier Universitaire de Nîmes, Nîmes, France (P.B.); Department of Hematology, Centre Hospitalier Universitaire de Caen, Caen, France (G.D., O.R.); Department of Hematology, Centre Henri Becquerel, Université de Rouen, INSERM U918, Rouen, France (F.J.); Department of Neurology, Centre Hospitalier Universitaire de Poitiers, Poitiers, France (D.L.); Department of Hematology and Cell Therapy, Centre Hospitalier Universitaire de Tours, CIC, INSERM U1415, Tours, France (E.G.); Department of Hematology, Centre Hospitalier Unive
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.
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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