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      Ovary involvement of diffuse large B-cell lymphoma

      case-report

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          Summary

          Background:

          Primary ovarian non-Hodgkin’s lymphoma (PONHL) is an uncommon entity; its pathology is usually diffuse large B cell lymphoma (DLBCL).

          Case Reports:

          We report 3 cases of ovary involvement of DLBCL, 1 of which rapidly developed to central nervous system involvement. Diagnosis and subsequent treatment are discussed and the literature on the origin, epidemiology, clinical presentation, diagnosis, treatment and prognosis of ovary lymphoma are reviewed. All patients were diagnosed as having DLBCL after ovary biopsy, and were subsequently given regular chemotherapy. Two of them obtained remission and 1 of them had central nervous system involvement.

          Conclusions:

          Ovary involvement of DLBCL is rare; prognosis is related to the overall clinical manifestation and some serum biomarkers. Diagnosis is established by ovary biopsy. Inaccurate or delayed diagnosis is often due to the lesions presenting as a mass resembling ovary cancer and may lead to poor outcome. Treatment regimen mainly consists of chemotherapy (CHOP) associated with rituximab. Intrathecal chemotherapy may play an important role in prevention of central nervous system involvement.

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          Most cited references16

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          Intravenous methotrexate as central nervous system (CNS) prophylaxis is associated with a low risk of CNS recurrence in high-risk patients with diffuse large B-cell lymphoma.

          The outcome of patients with systemic diffuse large B-cell lymphoma (DLBCL) had improved over the past decade with the addition of monoclonal antibody therapy. Unfortunately, approximately 5% of these patients still developed a secondary central nervous system (CNS) recurrence followed invariably by rapid death. This rate is substantially increased in patients with certain high-risk features. Although prophylaxis against CNS recurrence with either intrathecal or intravenous methotrexate is commonly used for such patients, to the authors' knowledge, there is no standard of care. Retrospectively evaluated was the role of high-dose systemic methotrexate combined with standard cyclophosphamide, doxorubicin, vincristine, and prednisone with rituximab (R-CHOP) chemotherapy to decrease CNS recurrence in high-risk patients. A total of 65 patients with DLBCL and CNS risk factors were identified at the study institution between 2000 and 2008 who received intravenous methotrexate as CNS prophylaxis concurrent with standard systemic therapy with curative intent. CNS recurrence rate, progression-free survival, and overall survival were calculated. Patients received a median of 3 cycles of methotrexate at a dose of 3.5 gm/m(2) with leucovorin rescue. The complete response rate was 86%, with 6% partial responses. At a median follow-up of 33 months, there were only 2 CNS recurrences (3%) in this high-risk population. The 3-year progression-free and overall survival rates were 76% and 78%, respectively. Complications associated with methotrexate therapy included transient renal dysfunction in 7 patients and a delay in systemic chemotherapy in 8 patients. Intravenous methotrexate can be safely administered concurrently with R-CHOP and is associated with a low risk of CNS recurrence in high-risk patients. © 2010 American Cancer Society.
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            National Cancer Institute sponsored study of classifications of non-Hodgkin's lymphomas: summary and description of a working formulation for clinical usage. The Non-Hodgkin's Lymphoma Pathologic Classification Project.

            An international multi-institutional clinicopathologic study of 1175 cases of non-Hodgkin's lymphoma sponsored by the National Institute has been completed. Histologic slides and clinical records were examined from previously untreated patients seen during the period between July 1971 and December 1975 at four institutions, three in the United States and one in Italy. The reproducibility and clinical relevance of the six major classifications of the non-Hodgkin's lymphomas was tested by six "expert" pathologists, each a proponent of a major classification, and six very experienced pathologists not identified with one of the major classifications. Immunologic methods were not employed in the study design. A summary of the methods employed and the conclusions of the study is described. The major conclusion was that all six classifications were valuable and comparable in reproducibility and clinical correlations. The clinical significance of a follicular architecture, independent of cell type was confirmed. A working formulation of non-Hodgkin's lymphomas is described which separates the disease into ten major types utilizing morphologic criteria only. Subtypes are also described which allow translation of all of the major classifications into comparable groups. Histologic criteria are presented for each major type and equivalent terms are given for each type in the six major classifications. The formulation is not proposed as a new classification but a means of translation among the various systems and to facilitate clinical comparisons of case reports and therapeutic trials. The report contains commentaries by five of the "expert" pathologists on the value and conclusions of this unique study.
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              Central nervous system (CNS) relapse in diffuse large B cell lymphoma (DLBCL): pre- and post-rituximab.

              Central nervous system (CNS)-directed prophylactic intrathecal (IT) therapy is indicated in patients with Burkitt and acute lymphoblastic lymphoma. Its role in diffuse large B cell lymphoma (DLBCL), a heterogeneous subtype, is less well defined. While addition of rituximab to standard cyclophosphamide-hydroxydaunorubicin-oncovin-prednisone (CHOP) chemotherapy (R-CHOP) has improved the outcomes of DLBCL patients, its role in reducing CNS relapse is unclear. We aim to (1) evaluate the clinical risk factors predictive of CNS relapse, (2) the role of rituximab in influencing CNS relapse, and (3) role of intrathecal prophylaxis. Four hundred ninety-nine patients with DLBCL from 2000 to 2008 were included (CHOP 179 vs. R-CHOP 320). IT prophylaxis was administered to 82 patients based on our institution's guidelines. Baseline characteristics between CHOP- and R-CHOP-treated patients were similar. Although R-CHOP significantly increased the complete remission rate from 71% to 81% (P 1; hazard ratio (HR) = 2.01, 95% confidence interval (CI) 1.29-3.14), failure to attain remission (non-complete response (CR) vs. CR: HR = 2.39, 95% CI = 1.03 to 5.51), testicular (HR = 6.67, 95% CI = 1.62 to 27.53), kidney (HR = 20.14, 95% CI = 5.23 to 77.46), and breast involvement (HR = 6.14, 95% CI = 1.61 to 23.37) were each independently predictive of CNS relapse. Use of IT prophylaxis did not appear to decrease CNS relapse. Median survival after CNS relapse was 3.2 months. CNS relapse, a fatal event, remains a challenge in R-CHOP-treated patients. IT prophylaxis may not be sufficient to reduce CNS relapse, and strategies including systemic agents with high CNS penetration should be evaluated in high-risk patients identified in this study.
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                Author and article information

                Journal
                Am J Case Rep
                Am J Case Rep
                amjcaserep
                The American Journal of Case Reports
                International Scientific Literature, Inc.
                1941-5923
                2012
                05 June 2012
                : 13
                : 96-98
                Affiliations
                Department of Hematology, Shengjing Hospital, China Medical University, China
                Author notes
                Author’s address: Wei Yang, Department of Hematology, Shengjing Hospital, China Medical University, China, e-mail: yangw@ 123456sj-hospital.org
                Article
                882997
                10.12659/AJCR.882997
                3616171
                23569499
                84db446a-1419-4c74-ad1b-6e1c4570c068
                © Am J Case Rep, 2012

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

                History
                : 07 January 2012
                : 10 May 2012
                Categories
                Case Report

                ovary,dlbcl-lymphoma,central nervous system (cns),intrathecal chemotherapy

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