Clément Desjardins1, Delphine Larrieu-Ciron1,2, Sylvain Choquet3, Karima Mokhtari4, Frédéric Charlotte5, Lucia Nichelli6, Bertrand Mathon7, Guido Ahle8, Magali Le Garff-Tavernier9, Andrea Morales-Martinez1, Caroline Dehais1, Khê Hoang-Xuan1, Caroline Houillier10. 1. Neuro-Oncology Department, Hôpital Pitié Salpêtrière, APHP, Sorbonne Université, IHU, ICM, Paris, France. 2. Neurology Department, Hôpital Pierre Paul Riquet, CHU de Toulouse Purpan, Toulouse, France. 3. Clinical Hematology Department, Hôpital Pitié-Salpêtrière, APHP, Paris, France. 4. Neuropathology Department, Hôpital Pitié-Salpêtrière, APHP, Paris, France. 5. Service d'Anatomie et Cytologie Pathologiques, Groupe Hospitalier Pitié-Salpétrière-Charles Foix, Sorbonne Université, Paris, France. 6. Neuroradiology Department, Hôpital Pitié-Salpêtrière, APHP, Paris, France. 7. Neurosurgery Department, Hôpital Pitié-Salpêtrière, APHP, Paris, France. 8. Department of Neurology, Hôpitaux Civils de Colmar, Colmar, France. 9. Hematobiology Department, Hôpital Pitié-Salpêtrière, APHP, Paris, France. 10. Neuro-Oncology Department, Hôpital Pitié Salpêtrière, APHP, Sorbonne Université, IHU, ICM, Paris, France. caroline.houillier@aphp.fr.
Abstract
BACKGROUND: Mucosae-associated lymphoid tissue (MALT) lymphomas are a rare and poorly understood form of primary central nervous system lymphoma (PCNSL). The aim of this study was to better describe these tumors, their management and their long-term prognosis. METHODS: Patients with primary CNS MALT lymphoma (PCNSML) were retrospectively selected from the database on PCNSL of the Pitié-Salpêtrière Hospital. RESULTS: Of 662 PCNSL, 11 (1.7%) PCNSML (9 females and 2 males, median age: 56 years) were selected. The median time from first symptoms to diagnosis was 13 months. Location was dural in 8 cases and parenchymal in 3 cases. The disease was multifocal/diffuse in 7 cases. In first line, all patients received chemotherapy (high-dose methotrexate (HD-MTX) based chemotherapy (n = 4) and non-HD-MTX-based chemotherapy (n = 7)), preceded by surgery in 4 cases. None received radiotherapy. According to the IPCG (International PCNSL Collaborative Group) criteria, the overall response rate was 7/11 (64%). At latest news, 5 patients had persistent contrast enhancement, stable with no treatment since a median of 57 months, raising the question of complete response despite persisting contrast enhancement. No patient developed neurotoxicity except for one patient who subsequently received radiotherapy. The median follow-up was 109 months. The median progression-free survival was 78.0 months and the 10-year overall survival rate was 90%. CONCLUSION: This is the largest series demonstrating that chemotherapy is an efficient treatment in PCNSML, with an excellent long-term outcome and the absence of neurotoxicity, and calling into question the relevance of the IPCG criteria for the evaluation of response.
BACKGROUND: Mucosae-associated lymphoid tissue (MALT) lymphomas are a rare and poorly understood form of primary central nervous system lymphoma (PCNSL). The aim of this study was to better describe these tumors, their management and their long-term prognosis. METHODS: Patients with primary CNS MALT lymphoma (PCNSML) were retrospectively selected from the database on PCNSL of the Pitié-Salpêtrière Hospital. RESULTS: Of 662 PCNSL, 11 (1.7%) PCNSML (9 females and 2 males, median age: 56 years) were selected. The median time from first symptoms to diagnosis was 13 months. Location was dural in 8 cases and parenchymal in 3 cases. The disease was multifocal/diffuse in 7 cases. In first line, all patients received chemotherapy (high-dose methotrexate (HD-MTX) based chemotherapy (n = 4) and non-HD-MTX-based chemotherapy (n = 7)), preceded by surgery in 4 cases. None received radiotherapy. According to the IPCG (International PCNSL Collaborative Group) criteria, the overall response rate was 7/11 (64%). At latest news, 5 patients had persistent contrast enhancement, stable with no treatment since a median of 57 months, raising the question of complete response despite persisting contrast enhancement. No patient developed neurotoxicity except for one patient who subsequently received radiotherapy. The median follow-up was 109 months. The median progression-free survival was 78.0 months and the 10-year overall survival rate was 90%. CONCLUSION: This is the largest series demonstrating that chemotherapy is an efficient treatment in PCNSML, with an excellent long-term outcome and the absence of neurotoxicity, and calling into question the relevance of the IPCG criteria for the evaluation of response.
Authors: Pang-Hsien Tu; Caterina Giannini; Alexander R Judkins; Jason M Schwalb; Richard Burack; Brian P O'Neill; Anthony T Yachnis; Peter C Burger; Bernd W Scheithauer; Arie Perry Journal: J Clin Oncol Date: 2005-07-11 Impact factor: 44.544
Authors: Mohammad O Khalil; Lindsay M Morton; Susan S Devesa; David P Check; Rochelle E Curtis; Dennis D Weisenburger; Graça M Dores Journal: Br J Haematol Date: 2014-01-12 Impact factor: 6.998