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      Primary Hepatic Lymphoma: A Challenging Diagnosis

      case-report

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          Abstract

          Introduction. Primary hepatic lymphoma is an unusual malignancy and is very difficult to diagnose promptly. An intrigue case presenting with cholestatic jaundice is reviewed and main disease characteristics are further discussed. Case Report. A 70-year-old male presented with dull right upper quadrant abdominal pain and mild cholestatic jaundice. Initial evaluation revealed mildly elevated liver function tests and normal tumor markers, while imaging with an abdominal CT-scan showed multiple hypodense nodules in both liver lobes. First impression of metastatic deposits from gastrointestinal origin was not confirmed by endoscopic means. After CT-guided biopsy, primary diffuse large B-cells non-Hodgkin lymphoma was revealed. Appropriate chemotherapy improved patient's condition markedly. Discussion. Primary hepatic lymphoma is a rare form of extranodal lymphomas, accounting for less than 1% of all extranodal lymphomas in general. In order to define the condition as PHL, liver has to be the only site of lymphoma occurrence or to be involved in a major degree with minimal nonliver disease. Most PHLs are of B-cell origin with large cells as the main cell type.

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          Primary non-Hodgkin's lymphoma of the liver.

          We review the literature on primary hepatic lymphoma (PHL). PHL is a rare malignancy, and constitutes about 0.016% of all cases of non-Hodgkin's lymphoma. It has been reported to occur with increased frequency in patients with chronic hepatitis C infection. Most patients with PHL present with abdominal pain, constitutional symptoms and have hepatomegaly on examination. Imaging studies reveal solitary, or less often, multiple masses in the liver. The predominant histology is B-cell lymphoma, most commonly diffuse large cell type. Most patients are treated with chemotherapy, with some physicians employing a multimodality approach incorporating surgery and radiotherapy with chemotherapy. The prognosis is variable, with good response to early aggressive combination chemotherapy.
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            Immunohistochemical prognostic markers in diffuse large B-cell lymphoma: validation of tissue microarray as a prerequisite for broad clinical applications--a study from the Lunenburg Lymphoma Biomarker Consortium.

            The results of immunohistochemical class prediction and prognostic stratification of diffuse large B-cell lymphoma (DLBCL) have been remarkably various thus far. Apart from biologic variations, this may be caused by differences in laboratory techniques, scoring definitions, and inter- and intraobserver variations. In this study, an international collaboration of clinical lymphoma research groups from Europe, United States, and Canada concentrated on validation and standardization of immunohistochemistry of the currently potentially interesting prognostic markers in DLBCL. Sections of a tissue microarray from 36 patients with DLBCL were stained in eight laboratories with antibodies to CD20, CD5, bcl-2, bcl-6, CD10, HLA-DR, MUM1, and MIB-1 according to local methods. The study was performed in two rounds firstly focused on the evaluation of laboratory staining variation and secondly on the scoring variation. Different laboratory staining techniques resulted in unexpectedly highly variable results and very poor reproducibility in scoring for almost all markers. No single laboratory stood out as uniformly poor or excellent. With elimination of variation due to staining, high agreement was found for CD20, HLA-DR, and CD10. Poor agreement was found for bcl-6 and Ki-67. Optimization of techniques and uniformly agreed on scoring criteria improved reproducibility. This study shows that semiquantitative immunohistochemistry for subclassification of DLBCL is feasible and reproducible, but exhibits varying rates of concordance for different markers. These findings may explain the wide variation of biomarker prognostic impact reported in the literature. Harmonization of techniques and centralized consensus review appears mandatory when using immunohistochemical biomarkers for treatment stratification.
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              Primary non-Hodgkin lymphoma of liver

              Primary non-Hodgkin lymphoma (nhl) of liver is a very rare malignancy. Here, we report the case of a 65-year-old man who presented with constipation and right groin pain of 2 months’ duration. A computed tomography (ct) scan of the abdomen incidentally detected multiple hypodense nodules in both lobes of the liver. Diagnosis of primary nhl of liver was made using ultrasound-guided biopsy. Extensive investigations—which included bone marrow biopsy; fluorescence in situ hybridization; flow cytometry; ct scan of chest, abdomen, and pelvis; and whole-body positron-emission tomography—showed no involvement of bone marrow, lymph nodes, spleen, or any other organ. The patient is currently being treated with a chop-r (cyclophosphamide–doxorubicin–vincristine–prednisolone/rituximab) regimen. The case has many unique features, including normal liver function tests, especially that for lactate dehydrogenase; no type B symptoms; and negative serology for viruses. The case demonstrates that primary hepatic lymphoma should be considered in the differential diagnosis of space-occupying liver lesions in presence of normal levels of alpha-fetoprotein and carcinoembryonic antigen. The literature is extensively reviewed.
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                Author and article information

                Journal
                Case Rep Oncol Med
                Case Rep Oncol Med
                CRIONM
                Case Reports in Oncological Medicine
                Hindawi Publishing Corporation
                2090-6706
                2090-6714
                2014
                11 May 2014
                : 2014
                : 212598
                Affiliations
                1Pathology Department, Tzaneion General Hospital, 185 36 Piraeus, Greece
                22nd Department of Surgery, University Hospital Aretaieion, 115 28 Athens, Greece
                31st Department of Surgery, Tzaneion General Hospital, 185 36 Piraeus, Greece
                Author notes

                Academic Editor: Xavier G. Thomas

                Article
                10.1155/2014/212598
                4037615
                ed772af5-3663-431c-af36-5fe8e7f6fc3a
                Copyright © 2014 D. Myoteri et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 2 February 2014
                : 28 April 2014
                Categories
                Case Report

                Oncology & Radiotherapy
                Oncology & Radiotherapy

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