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      Primary extranodal natural killer/T‐cell lymphoma of bronchus and lung: A case report and review of literature

      case-report

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          Abstract

          Primary pulmonary non‐ Hodgkin's lymphoma ( NHL) is very rare. It represents less than 1% of all NHL, and 0.5–1% of all primary pulmonary malignancies. Almost all cases of primary pulmonary NHL originate from B‐cell lineage. We present a case of a 53‐year‐old man with primary extranodal NK/ T‐cell lymphoma of the bronchus and lung, presented progressive dyspnea caused by right lower lung consolidation, and pleural effusion. Initial chest computed tomography suggested advanced lung cancer. Bronchofiberscopy showed a polypoid tumor on which a biopsy was performed. Histologically, the diffusely infiltrative atypical cells were positive for cytoplasmic CD3, CD56, granzyme B, and negative for cytokeratin, CD20 immunostains, suggesting NK/ T cell lineages. In situ hybridization for Epstein‐ Barr virus encoded ribonucleic acid ( EBER) was positive. Herein, we discuss the clinicopathological features of this case and review the literature on primary extranodal NK/ T‐cell lymphoma of the lung. Compared with other patients, who died after the first cycle of chemotherapy and/or within three months, our patient had longer survival under aggressive chemotherapy and auto‐peripheral blood stem cell transplantation.

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          Most cited references14

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          SMILE for natural killer/T-cell lymphoma: analysis of safety and efficacy from the Asia Lymphoma Study Group.

          Natural killer/T-cell lymphoma is rare and aggressive, with poor outcome. Optimal treatment remains unclear. A novel regimen dexamethasone, methotrexate, ifosfamide, l-asparaginase, and etoposide (SMILE) showed promise in phase 1/2 studies with restrictive recruitment criteria. To define the general applicability of SMILE, 43 newly diagnosed and 44 relapsed/refractory patients (nasal, N = 60, nonnasal, N = 21; disseminated, N = 6; male, N = 59; female, N = 28) at a median age of 51 years (23-83 years) were treated. Poor-risk factors included stage III/IV disease (56%), international prognostic index of 3 to 5 (43%), and Korean prognostic scores of 3 to 4 (41%). A median of 3 (0-6; total = 315) courses of SMILE were administered. Significant toxicities included grade 3/4 neutropenia (N = 57; 5 sepsis-related deaths); grade 3/4 thrombocytopenia (N = 36); and nephrotoxicity (N = 15; 1 acute renal failure and death). Interim analysis after 2 to 3 cycles showed complete remission rate of 56%, partial remission rate of 22%, giving an overall response rate of 78%. On treatment completion, the overall-response rate became 81% (complete remission = 66%, partial remission = 15%). Response rates were similar for newly diagnosed or relapsed/refractory patients. At a median follow-up of 31 months (1-84 months), the 5-year overall survival was 50% and 4-year disease-free-survival was 64%. Multivariate analysis showed that international prognostic index was the most significant factor impacting on outcome and survivals.
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            Primary pulmonary lymphoma.

            Three distinct entities are now covered by the definition of primary pulmonary clonal lymphoid proliferation. The aim of this review is to describe the pathophysiological, diagnostic, prognostic and therapeutic aspects of these three entities. Low-grade pulmonary B-cell lymphoma is the most frequent form of primary pulmonary clonal lymphoid proliferation. It arises from mucosa-associated lymphoid tissue. It is usually indolent and appears in the form of a chronic alveolar opacity. The prognosis is excellent, but treatment is controversial (simple monitoring, surgery or single-agent chemotherapy). High-grade pulmonary B-cell lymphoma is far rarer and usually occurs in individuals with an underlying disorder (e.g. immunodeficiency). The prognosis is poor and therapeutic options depend on the underlying disorder. The inclusion of lymphomatoid granulomatosis in the definition of primary pulmonary lymphomas is controversial. The clonal nature of the proliferation is very rarely demonstrated and extrapulmonary involvement is frequent (upper airways, skin, kidneys, central nervous system, etc.). The prognosis is extremely variable, with some authors reporting complete remission with steroids and cyclophosphamide and others reporting failure of combination chemotherapy.
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              Pathologic and clinical features of primary pulmonary extranodal marginal zone B-cell lymphoma of MALT type.

              We reviewed pathologic, phenotypic, and clinical features of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) type primarily involving lung to address unresolved questions regarding behavior and pathologic features of unambiguously diagnosed pulmonary MALT lymphoma. Lung specimens from 50 patients were reviewed. Forty-one had low-grade MALT lymphoma. Nine had low-grade MALT lymphoma and diffuse large B-cell lymphoma. The patients included 32 women and 18 men with a median age of 68 years (range 34-88 years). Half of the patients were asymptomatic at the time lymphoma was diagnosed. Radiographic abnormalities were more commonly unilateral (37 patients) than bilateral (12 patients). Localized masses or nodules occurred in 39 patients. Associated autoimmune disorders (29%) and monoclonal gammopathies (43%) were common. Low-grade lymphomas formed intraparenchymal masses composed of centrocyte-like cells, plasmacytoid lymphocytes, and plasma cells that formed lymphoepithelial lesions and exhibited a lymphangitic growth pattern. Mediastinal lymph nodes were involved histologically in 44% of cases. Lymphoma-specific survival was 71.7% at 10 years, and overall survival was significantly worse than age-and gender-matched control patients. None of the following features predicted those patients who had an adverse outcome: systemic symptoms, presence of autoimmune disorders or paraproteinemia, anatomic distribution and number of pulmonary lesions, lymph node involvement, or presence of anthracycline-treated large B-cell lymphoma.
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                Author and article information

                Journal
                Thorac Cancer
                Thorac Cancer
                10.1111/(ISSN)1759-7714
                TCA
                Thoracic Cancer
                John Wiley and Sons Inc. (Hoboken )
                1759-7706
                1759-7714
                19 March 2015
                January 2016
                : 7
                : 1 ( doiID: 10.1111/tca.2016.7.issue-1 )
                : 140-144
                Affiliations
                [ 1 ] Department of Pathology and Laboratory MedicineVeterans General Hospital‐ Kaohsiung Kaohsiung Taiwan
                [ 2 ] Department of Internal Medicine, Chest DivisionVeterans General Hospital‐ Kaohsiung Kaohsiung Taiwan
                Author notes
                [*] [* ] Correspondence

                Pin‐Pen Hsieh, Department of Pathology and Laboratory Medicine, Veterans General Hospital‐Kaohsiung, Taiwan, 386 Ta‐Chung 1st. Rd, Kaohsiung, Taiwan 813.

                Tel: +886 7 3422121 #6306

                Fax: +886 7 3468317

                Email: pinpenh@ 123456gmail.com

                Article
                TCA12254
                10.1111/1759-7714.12254
                4718123
                26816549
                82f6251a-9d92-4df0-8123-72e7e65f28e2
                © 2015 The Authors. Thoracic Cancer published by China Lung Oncology Group and Wiley Publishing Asia Pty Ltd.

                This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.

                History
                : 23 January 2015
                : 16 February 2015
                Page count
                Pages: 5
                Categories
                Case Reports
                Case Report
                Custom metadata
                2.0
                tca12254
                January 2016
                Converter:WILEY_ML3GV2_TO_NLMPMC version:4.7.5 mode:remove_FC converted:19.01.2016

                lymphoma,lung and/or bronchus,natural killer/t‐cell

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