Brain lymphoma, LPSNC, cerebral eye lymphoma
Cerebral lymphoma, frequent peri-ventricular localization
Frequent localization as with the above MRI, the most common form is a periventricular lesion, central gray nuclei or the callus body, unique, taking the contrast in an intense and nodular manner with little edema and a mass effect.
Lymphoma The lymphoma is a disease of lymphocytes, small cells with rounded and bulky nuclei, of the immune system. Lymphocytes are of two orders, T, T4 (auxiliary) and T8 (killers), learning and maturation in the thymus and B (of English bone, bone or purse of Fabricius), maturing almost entirely in the bone marrow. B lymphocytes are the memory of the immune system and immunocompetent as soon as they are released from the bone. The mode of action of T is the direct recognition of the foreign cell, the bacterium or the cancer cell in comparison with its data bank received during its maturation. T and B work together to eliminate the foreign body. After recognizing a foreign body, B can only manufacture antigens against this alien. Lymphoma can occur in several areas of the body, including lymph nodes, spleen, liver, digestive tract and brain. When lymphoma touches the brain and spinal cord (central nervous system), symptoms can be headaches, confusion, memory loss, convulsions, speech problems and decreased body capacity. Primary brain lymphoma is a rare tumor, fairly close to the glioblastoma in MRI imaging. It is sometimes found in immunocompromised patients (organ transplant recipients, HIV +, for example). A secondary brain lymphoma tumor usually manifests in the form of an invasion of the brain envelopes, a leptoméningée impairment. If lymphoma also touches the eye, we are talking about cerebral eye lymphoma.
Treatments
High-dose methotrexate (MTX) chemotherapy (3.5 g/m2) and radiotherapy. This treatment now allows healing.
Prognosis
Currently any newly diagnosed patient must be registered in the LOC network. Recovery is now taking place in many patients while the average survival was only 3-4 years with a significant long-term survivor rate (20-30%).
Primary brain lymphoma and central nervous system LCP, large cell B lymphoma
Large-cell Diffuse B lymphoma is a subtype of non-Hodgkin's lymphoma most common in adults, with a median age of 65 years (60 years-70 years) initially manifested by a single or multiple mass with rapid growth, painful or not, with A nodal and extra-nodal localization (thyroid, skin, breast, gastrointestinal tract, testicles, bone or brain), and may be accompanied by fever, night sweats and weight loss. Large-cell Diffuse B lymphoma has an aggressive evolution, with older patients having a worse prognosis than younger patients. Recurrences are common. Large-cell Diffuse B lymphoma is the most common subtype of non-Hodgkin's lymphoma in adults, with a median age of presentation in the sixth decade (more rarely in adolescents and children), initially manifested in a Fast growing single or multiple mass (painful or not) of nodal and extra-nodal localization (thyroid, skin, breast, gastrointestinal tract, testicles, bone or brain), and may be accompanied by fever, night sweats and Weight loss. Large-cell Diffuse B lymphoma has an aggressive evolution, with older patients having a worse prognosis than younger patients. Recurrences are common. LCP primary cerebral lymphomas are intracerebral lymphoma tumours occurring in a patient without history of non-Hodgkin's lymphoma and with no other lymphoma locations outside the central nervous system, Brain or eye. This is 80% of the cases of large-cell diffuse malignant B-type lymphoma (REAL classification). The existence of a immunosuppression is a factor favouring it being congenital or acquired (AIDS, immunosuppressive therapy for grafting or sickness. Patients with a competent immune system must be included since 2012 in the program validated by the cancer plan by GFME LOC. The LOC centres are numbered 19 and the list is available here with the manager.
LOC Centers
For example, for Rennes, we must contact the LOC Manager, Professor Thierry Lamy de la Chapelle at the hematology Department of the CHU de Pontchaillou at 02 99 28 43 21.
High dose chemotherapy of Methotrexate effective 71st dossier ASCO 2009 2070-characteristics and results of elderly patients with central CNS nervous system lymphoma (PCNSL or LPSNC).
Citation: J Clin Oncol 27:15s, 2009 (suppl; Abstr 2070)
Author (s): D. E. Ney, A. S. Reiner, H. D. Skinner, K. S. medleys, L. M. DeAngelis, L. E. Abrey; Memorial Sloan-Kettering Cancer Center, New York, NY the frequency of PCNSL increases and is highest in patients ≥ 65 years of age. Systemic chemotherapy (CT), radiotherapy ± (RT) improves survival, but treatment leads to greater toxicity in this population. The optimal treatment must still be determined. The purpose of this study was to characterize patients ≥ 65 years with PCNSL in our institution and to identify the results in treatment. We have identified patients ≥ 65 years treated for PCNSL from 1986 to 2008. The tables were examined for demography, treatment detail, tumor progression, and survival.
174 patients were identified with a median age of 72 years (range: 65-89 years). 60% of patients had a stereotactic biopsy for diagnosis. 93% had histological or cytological diagnosis. 14 patients had evidence of systemic involvement. 82% of patients were treated with Methotrexate at high doses (3, 5g/m2), and 13% did not receive CT chemotherapy. Of the patients who received CT, 76% had a radiographic response (CR + PR), 3% had the disease stable while 12% progressed. Only 26% had RT as initial therapy. The complete estimated CR response at the end of the therapy was 67%, eventually, 52% of the patients relapsed. The median time to progression was 24 months (range: 1-91 months). Of the patients who relapsed, 85% received rescue therapy consisting of CT (n = 42), RT (n = 14), or both (n = 7) while 15% received no additional treatment. 48% of patients had a CR or partial PR response at the end of the therapy while 26% had PD progression, the remainder not being evaluated. Median total survival for the entire cohort was 25 months (range: 0.5 to 177 + months) with 3-year survival of 36%. 17% developed late-treatment-related neurological toxicity. RT Administration has been associated with the development of neurotoxicity. 39 patients remain alive after a median follow-up of 34 months (range: 0.5-177 months). Elderly patients may receive an aggressive chemotherapy regimen with good results.
Intensive chemotherapy and immunotherapy in patients with newly diagnosed nerve system primary lymphoma: CALGB 50202 (Alliance 50202) James L. Rubenstein and Lawrence D. Kaplan, Helen Diller Comprehensive Cancer Centre, University of California, San Francisco, Eric D. Hsi and Megan O. Nakashima, Cleveland Clinic, OH,; Jeffrey L. Johnson and Sin-Ho as Jung, Duke Cancer Center, Durham, NC, Barbara Grant, University of Vermont, Burlington, VT; and Bruce D. Cheson, Georgetown University Hospital, Washington, DC.
Abstract the neurocognitive toxicity of whole brain radiotherapy (WBRT) has motivated the development of alternatives by dose-intensive chemotherapy strategies as a consolidation of central nerve system (PCNSL) lymphomas. A multicentre, high-dose consolidation trial, without WBRT radiotherapy, was performed in PCNSL. The objectives were to determine a complete response rate (CR) after methotrexate therapy, Temozolomide (Temodal), and Rituximab (MT-R), the feasibility of a 2nd approach using a high dose consolidation with Etoposide plus Cytarabine (EA), Progression-free survival (PFS) and finally the correlation between clinical and molecular prognostic factors and results. 44 patients with newly diagnosed PCNSL have been treated with MT-R, and the patients who have completed CR have received EA consolidation. We performed a multifactorial analysis of molecular prognostic biomarkers in PCNSL as part of a clinical trial. The rate of CR at MT-R was 66%. The PFS at 2 years was 57%, with a median follow-up of 4.9 years. The rate of progression at 2 years was 59% and for patients who completed the consolidation, it was 77%. Patients over 60 years of age had comparable results to the youngest and the most important clinical prognostic variable was the time of treatment. A high expression of BCL6 corresponded with a shorter survival. CALGB 50202 demonstrates for the first time to our knowledge that dose-intensive consolidation for PCNSL is feasible and allows for non-progression or total survivals comparable to WBRT. Following these encouraging results, an intergroup clinical trial was activated to compare EA consolidation with myéloablative chemotherapy for PCNSL against an arm with WBRT.
The LOC network with POLA, 2 validated cancer plan programs
The LOC network for the treatment of eye-cerebral lymphomas for immuno-competent patients set up early 2012 as part of the cancer plan
2 national Centres Pity Salpêtrière and the Hôpital René Hayward-Institut Curie and 20 regional experts centre throughout France ensures harmonisation of the treatments of eye lymphomas of immuno-competent patients. There are 300 new cases of primitive lymphoma of the central nervous system LPSNC, in France, each year, or 5% of the 6000 people diagnosed with a primitive brain tumor. Patients can now apply for the LOC project.
For information on the management of eye lymphomas of immunocompetent patients, our special page.
A marker of disease
72nd Dossier Asco 2009 2071-CXCL13 and CXCL12 in the central nervous system (CNS) for patients with lymphoma.
Citation: J Clin Oncol 27:15s, 2009 (suppl; Abstr 2071)
Author (s): J Clin Oncol 27:15s, 2009 (suppl; abstr 2071) CNS Malignant lymphocytes may play a role in the pathogenesis of CNS lymphomas. Recently, the expression of chemokine CXCR4 and CXCR5 receptors as well as their ligands, the CXCL12 and CXCL13 chemokines of the tumor cells of the primary CNS lymphoma (PCNSL) have been demonstrated. In this study, we evaluated CXCL12 and CXCL13 in the cerebrospinal fluid (CSF) and serum of patients with CNS lymphoma. Samples of 30 Patients with CNS lymphoma (23 with PCNSL and 7 with secondary CNS lymphoma) and 40 diseased controls (10 patients with other CNS malignancies and 30 without malignant CNS disease) were examined. The concentrations of CXCL12 and CXCL13 were measured using ELISA. The grade of defect in the blood/brain barrier (BBB) was estimated by the CSF/serum albumin ratio. Controls in patients with CNS lymphoma did not differ in CXCL12 serum and CSF. The level in the CXCL13 serum was generally low. However, CXCL13 levels in CSF were only high in patients with CNS lymphomas but not in controls. Chemokine levels in CSF and serum were not equivalent. In CNS lymphoma the concentration of CXCL13 in the CSF corresponded with the disturbance of the BBB blood/brain barrier. High levels in the CSF of CXCL12 and CXCL13 measured in 7 patients with CNS lymphoma decreased in 5 patients who responded to chemotherapy, and increased in 2 in progression of lymphoma. Our results suggest a CXCL13 production in patients with CNS lymphoma that decreases in response to therapy. Therefore, CXCL13 can represent a marker for additional diagnostic and prognostic studies.
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