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March 10, 2020; 94 (10) Article

Management and outcome of primary CNS lymphoma in the modern era

An LOC network study

Caroline Houillier, Carole Soussain, Hervé Ghesquières, Pierre Soubeyran, Olivier Chinot, Luc Taillandier, Thierry Lamy, Sylvain Choquet, View ORCID ProfileGuido Ahle, Gandhi Damaj, Philippe Agapé, Cécile Moluçon-Chabrot, Alexandra Amiel, Vincent Delwail, Michel Fabbro, Fabrice Jardin, Adrien Chauchet, Marie-Pierre Moles-Moreau, Franck Morschhauser, Olivier Casasnovas, Rémy Gressin, Luc-Matthieu Fornecker, Julie Abraham, Jean-Pierre Marolleau, Adrian Tempescul, Chantal Campello, Philippe Colin, Jérôme Tamburini, Kamel Laribi, Caroline Serrier, Corinne Haioun, Safia Chebrek, Anna Schmitt, Marie Blonski, Roch Houot, View ORCID ProfileEileen Boyle, Jacques-Olivier Bay, Lucie Oberic, Emeline Tabouret, Agathe Waultier, Nadine Martin-Duverneuil, View ORCID ProfileValérie Touitou, Nathalie Cassoux, View ORCID ProfileAurélie Kas, Karima Mokhtari, Frederic Charlotte, Agusti Alentorn, Loïc Feuvret, Magali Le Garff-Tavernier, Myrto Costopoulos, View ORCID ProfileBertrand Mathon, Matthieu Peyre, Daniel Delgadillo, Hassen Douzane, Diane Genet, Bachir Aidaoui, Khê Hoang-Xuan, Emmanuel Gyan
First published January 6, 2020, DOI: https://doi.org/10.1212/WNL.0000000000008900
Caroline Houillier
From Service de Neurologie 2-Mazarin, Sorbonne Université, IHU, ICM (C. Houillier, A.A., D.D., H.D., D.G., B.A., K.H.-X.), Service d'Hématologie (S. Choquet), Service de Neuro-Radiologie (N.M.-D.), Service d'Ophtalmologie (V.T.), Service de Médecine Nucléaire (A.K.), Service de Neuro-Pathologie (K.M.), Service d'Anatomie et Cytologies Pathologiques (F.C.), Service de Radiothérapie (L.F.), Service d'Hémato-Biologie (M.L.G.-T., M.C.), and Service de Neurochirurgie (B.M., M.P.), APHP, Groupe Hospitalier Pitié-Salpêtrière, Paris; Service d'Hématologie (C. Soussain), Institut Curie, Site Saint-Cloud; Service d'Hématologie (H.G.), CHU Lyon Sud; Service d'Hématologie (P.S., A.S.), Institut Bergonié, Bordeaux; Service de Neuro-Oncologie (O. Chinot), Aix-Marseille Université, CNRS, INP, AP-HM, CHU de la Timone, Marseille; Service de Neurologie (L.T., M.B.), CHU de Nancy; Service d'Hématologie (R.H.), Inserm U1236 Université de Rennes 1 (T.L.), CHU de Rennes; Service de Neurologie (G.A.), Hôpitaux Civils, Colmar; Service d'Hématologie (G.D.), CHU de Caen; Service d'Oncologie Médical (P.A.), Institut de Cancérologie de l'Ouest, Saint Herblain; Service d'Hématologie (C.M.-C.), CHU de Clermont-Ferrand; Service de Neurologie (A.A.), CHU de Toulouse; Service d'Oncologie Hématologique et de Thérapie Cellulaire (V.D.), CHU de Poitiers, INSERM, CIC 1402, Centre d'Investigation Clinique, Université de Poitiers; Service d'Oncologie Médicale (M.F.), Institut du Cancer de Montpellier Val d'Aurelle; Service d'Hématologie (F.J.), Centre Henri Becquerel, Rouen; Service d'Hématologie (A.C.), CHU de Besançon; Service d'Hématologie (M.P.M.-M.), CHU d'Angers; Service d'Hématologie (F.M., E.B.), CHRU de Lille; Service d'Hématologie (O. Casasnovas), CHU de Dijon; Service d'Onco-Hématologie (R.G.), CHU de Grenoble; Service d'Hématologie (L.M.F.), CHU de Strasbourg; Service d'Hématologie (J.A.), CHU de Limoges; Service d'Hématologie (J.-P.M.), CHU d'Amiens; Service d'Hématologie (A.T.), CHU de Brest; Service de Neurologie (C.C.) and Service d'Hématologie (A.W.), CHU de Nîmes; Clinique Courlancy (P.C.), Reims; Service d'Hématologie (J.T.), Hôpital Cochin, APHP, Paris; Service d'Hématologie Clinique (K.L.), Centre Hospitalier, Le Mans; Service d'Hématologie (C. Serrier), Centre Hospitalier de Perpignan; Service d'Hématologie (C. Haioun), Hôpital Henri Mondor, Créteil, APHP; Service d'Hématologie Clinique (S. Chebrek), Centre Hospitalier d'Avignon; Service d'Hématologie (J.O.B.), CHU de Clermont-Ferrand; Service d'Hématologie (L.O.), Institut Universitaire du Cancer de Toulouse; Service de Neuro-Oncologie (E.T.), Aix-Marseille Univ, CNRS, INP, AP-HM, CHU de la Timone; Service d'Ophtalmologie (N.C.), Institut Curie, Université Paris V Descartes et PSL (Paris Science et Lettre), Paris; and Service d'Hématologie et Thérapie Cellulaire (E.G.), Centre d'Investigations Cliniques INSERM U1517, Centre Hospitalier Universitaire, Université de Tours, France.
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Carole Soussain
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Management and outcome of primary CNS lymphoma in the modern era
An LOC network study
Caroline Houillier, Carole Soussain, Hervé Ghesquières, Pierre Soubeyran, Olivier Chinot, Luc Taillandier, Thierry Lamy, Sylvain Choquet, Guido Ahle, Gandhi Damaj, Philippe Agapé, Cécile Moluçon-Chabrot, Alexandra Amiel, Vincent Delwail, Michel Fabbro, Fabrice Jardin, Adrien Chauchet, Marie-Pierre Moles-Moreau, Franck Morschhauser, Olivier Casasnovas, Rémy Gressin, Luc-Matthieu Fornecker, Julie Abraham, Jean-Pierre Marolleau, Adrian Tempescul, Chantal Campello, Philippe Colin, Jérôme Tamburini, Kamel Laribi, Caroline Serrier, Corinne Haioun, Safia Chebrek, Anna Schmitt, Marie Blonski, Roch Houot, Eileen Boyle, Jacques-Olivier Bay, Lucie Oberic, Emeline Tabouret, Agathe Waultier, Nadine Martin-Duverneuil, Valérie Touitou, Nathalie Cassoux, Aurélie Kas, Karima Mokhtari, Frederic Charlotte, Agusti Alentorn, Loïc Feuvret, Magali Le Garff-Tavernier, Myrto Costopoulos, Bertrand Mathon, Matthieu Peyre, Daniel Delgadillo, Hassen Douzane, Diane Genet, Bachir Aidaoui, Khê Hoang-Xuan, Emmanuel Gyan
Neurology Mar 2020, 94 (10) e1027-e1039; DOI: 10.1212/WNL.0000000000008900

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Abstract

Objective Real-life studies on patients with primary CNS lymphoma (PCNSL) are scarce. Our objective was to analyze, in a nationwide population-based study, the current medical practice in the management of PCNSL.

Methods The French oculo-cerebral lymphoma network (LOC) database prospectively records all newly diagnosed PCNSL cases from 32 French centers. Data of patients diagnosed between 2011 and 2016 were retrospectively analyzed.

Results We identified 1,002 immunocompetent patients (43% aged >70 years, median Karnofsky Performance Status [KPS] 60). First-line treatment was high-dose methotrexate-based chemotherapy in 92% of cases, with an increasing use of rituximab over time (66%). Patients <60 years of age received consolidation treatment in 77% of cases, consisting of whole-brain radiotherapy (WBRT) (54%) or high-dose chemotherapy with autologous stem cell transplantation (HCT-ASCT) (23%). Among patients >60 years of age, WBRT and HCT-ASCT consolidation were administered in only 9% and 2%, respectively. The complete response rate to initial chemotherapy was 50%. Median progression-free survival was 10.5 months. For relapse, second-line chemotherapy, HCT-ASCT, WBRT, and palliative care were offered to 55%, 17%, 10%, and 18% of patients, respectively. The median, 2-year, and 5-year overall survival was 25.3 months, 51%, and 38%, respectively (<60 years: not reached [NR], 70%, and 61%; >60 years: 15.4 months, 44%, and 28%). Age, KPS, sex, and response to induction CT were independent prognostic factors in multivariate analysis.

Conclusions Our study confirms the increasing proportion of elderly within the PCNSL population and shows comparable outcome in this population-based study with those reported by clinical trials, reflecting a notable application of recent PCNSL advances in treatment.

Glossary

CI=
confidence interval;
CR=
complete response;
CRu=
unconfirmed complete response;
HCT-ASCT=
high-dose chemotherapy with autologous stem cell transplantation;
HD-MTX-CT=
high-dose methotrexate-based chemotherapy;
IELSG=
International Extranodal Lymphoma Study Group;
KPS=
Karnofsky Performance Status;
LDH=
lactate dehydrogenase;
LOC=
French oculo-cerebral lymphoma network;
MSKCC=
Memorial Sloan Kettering Cancer Center;
ORR=
objective response rate;
OS=
overall survival;
PCNSL=
primary CNS lymphoma;
PFS=
progression-free survival;
PR=
partial response;
WBRT=
whole-brain radiotherapy

Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • ↵* These authors contributed equally to this work.

  • Received April 12, 2019.
  • Accepted in final form September 5, 2019.
  • © 2020 American Academy of Neurology
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