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Extranodal Sites (extranodal + site)
Selected AbstractsRosai,Dorfman disease presenting as a solitary mediastinal massPATHOLOGY INTERNATIONAL, Issue 4 2009Akira I. Hida Rosai,Dorfman disease (RDD) involving an extranodal site is a diagnostic challenge. Reported herein is the case of a 67-year-old man who presented with a solitary superior mediastinal mass. The lesion was clinically suspected of malignancy including lymphoma because of its high uptake during a 67Ga-scintigram and 18F-fluorodeoxyglucose,positron emission tomography. There was no evidence of spread of the disease. Histology of thoracoscopic biopsy specimens indicated granulomatous lesion with infiltration of lymphocytes, plasma cells, and histiocytes with lymphocytes engulfed in their cytoplasm. The lesion did not contain lymph node or thymic elements. On immunohistochemistry the histiocytes were positive for S-100 protein, CD68, and CD163 but were negative for CD1a. These findings suggested a diagnosis of RDD. Despite lack of intervention, the lesion remained almost the same size for 3 years. To the best of the authors' knowledge this is the first case of RDD presenting as a solitary mediastinal mass. [source] Clinical relevance of three subtypes of primary sinonasal lymphoma characterized by immunophenotypic analysisHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 7 2004Gwi Eon Kim MD Abstract Background. The purpose of this study was to investigate the clinical relevance of subtypes categorized by immunophenotypic analysis in primary sinonasal lymphomas. Methods. Eighty patients with localized non-Hodgkin's lymphoma involving the nasal cavity and/or paranasal sinuses were divided into three subtypes on the basis of their immunohistochemical findings: (A) B-cell lymphoma (n = 19), (B) T-cell lymphoma (n = 27), and (C) natural killer (NK)/T-cell lymphoma (n = 34). The clinicopathologic profiles, immunophenotypic data, patterns of treatment failure, and survival data among the three patient groups were retrospectively compared. Results. The nasal cavity was the predominant site of involvement in T-cell and NK/T-cell lymphoma, whereas sinus involvement without nasal disease was common in B-cell lymphoma. Systemic B symptoms were frequently observed in NK/T-cell lymphoma. Almost all patients with NK/T-cell lymphoma showed a strong association with the Epstein-Barr virus by in situ hybridization studies. Sixty-five patients (81%) patients achieved complete remission after initial treatment, but 36 (55%) of these subsequently experienced treatment failure. Although there were no significant differences in locoregional failure rates among the patients of the three groups, distant failure was far more common in B-cell or NK/T-cell lymphoma than in T-cell lymphoma (p = .005). Most B-cell lymphoma cases showed a predilection for sites of systemic failure in the nodal and extranodal sites below the diaphragm, such as the paraaortic lymph nodes or the gastrointestinal (GI) tract, whereas patients with NK/T-cell lymphoma showed an increased risk of systemic dissemination to the skin, testes, or GI tract, including the development of hemophagocytic syndrome. The 5-year actuarial and disease-free survival rates for all patients were 57% and 51%, respectively. Of the three subtypes of primary sinonasal lymphomas, T-cell lymphoma seemed to carry the most favorable prognosis and NK/T-cell lymphoma the worst. (The 5-year actuarial survival rate was 57% for B-cell lymphoma, 80% for T-cell lymphoma, 37% for NK/T-cell lymphoma; p = .02, log-rank.) By univariate and multivariate analyses, immunophenotype was identified as the most important prognostic factor. Conclusions. Our data indicate that the three subtypes of primary sinonasal lymphomas classified by immunohistochemical studies exhibit different clinical profiles, different patterns of failure, and different treatment outcomes. Given these observations, it is concluded that the recognition of these distinct subsets, diagnosed on the basis of immunophenotypic study, is very important and clinically relevant in predicting their potential behavior and prognosis. © 2004 Wiley Periodicals, Inc. Head Neck26: 584,593, 2004 [source] Central nervous system dissemination in immunocompetent patients with aggressive lymphomas: incidence, risk factors and therapeutic optionsHEMATOLOGICAL ONCOLOGY, Issue 2 2009Andrés J. M. Ferreri Abstract Central nervous system (CNS) dissemination is a rare (4,5%) but usually fatal complication of aggressive lymphomas. Prophylaxis modalities to prevent CNS dissemination in aggressive lymphomas cannot be widely applied to every lymphoma patient since it is associated with increased risk of neurotoxicity. Therefore, identification of high-risk patients as the best candidates to receive CNS prophylaxis constitutes a major endpoint in the management of these malignancies. Various risk factors and models for CNS recurrence have been described. Parameters reflecting the extent and proliferation of the disease, like elevated serum lactate dehydrogenase levels, involvement of multiple extranodal sites, advanced stage and high age-adjusted International Prognostic Index (IPI) score, as well as the involvement of specific anatomic sites, like testes, orbit, paranasal sinuses, have been identified and confirmed as important to predict CNS dissemination. Management of this complication in aggressive lymphomas with conventional-dose chemotherapy is associated with disappointing results, while some preliminary but encouraging experiences suggest a potential role of high-dose chemotherapy and stem cell transplantation. The analysis of recent clinical studies could lead to advancement in the prognosis of aggressive lymphomas, but several questions regarding the optimum chemotherapy combination, the best conditioning regimen and the role of radiation therapy and intrathecal chemotherapy remain still unanswered. The purposes of the present review are to critically analyse current data on the risk of CNS dissemination in aggressive lymphomas, the clinical presentation of secondary CNS lymphomas and the efficacy of CNS prophylaxis as well as to discuss the available therapeutic options for this devastating event. Copyright © 2009 John Wiley & Sons, Ltd. [source] BCL2 gene abnormalities define distinct clinical subsets of follicular lymphomaHISTOPATHOLOGY, Issue 3 2006J R Goodlad Aims:, Follicular lymphoma (FL) arising primarily in the skin has recently been proposed as a distinct entity on the basis of a low incidence of t(14;18)(q32;q21) and bcl-2 expression, with a very high percentage of patients surviving more than 5 years. However, cases of t(14;18)(q32;q21)-positive primary cutaneous FL (PCFL) and examples of t(14;18)(q32;q21)-negative FL at nodal and other extranodal sites, are well documented. The aim of this study was to test the hypothesis that there is a subtype of FL lacking t(14;18)(q32;q21), which preferentially involves certain sites but is not restricted by anatomical location. Methods and results:, A cohort of 47 stage 1 FL was stratified according to the presence or absence of t(14;18)(q32;q21) using conventional cytogenetics, polymerase chain reaction and interphase fluorescence in situ hybridization. Compared with t(14;18)(q32;q21)-positive cases, FL lacking the translocation were less likely to express CD10 or bcl-2 (P < 0.01), made up a significantly greater proportion of cases arising at extranodal sites (P < 0.001) and had a significantly better overall and disease-specific 5-year survival (P < 0.01). Conclusions:, These results support the concept of a subtype of FL lacking t(14;18)(q32;q21), characterized by low-intensity bcl-2 expression, a predilection for extranodal sites, particularly the skin, and a more favourable outcome than t(14;18)(q32;q21)-positive FL. [source] ,, T-cell lymphomas: a homogeneous entity?HISTOPATHOLOGY, Issue 4 2000De Wolf-Peeters ,, T-cells comprise an immunologically distinct lymphoid population, characterized by specific morphological, phenotypical and functional properties. Therefore it seems reasonable to speculate that neoplasms derived from this particular T-cell subset display distinct features. Indeed, the prototype ,, T-cell lymphoma, hepatosplenic T-cell lymphoma constitutes a unique clinicopathological entitity which is intimately associated with a ,, T-cell phenotype. However, ,, T-cell lymphomas have also been described in other extranodal sites where, unlike reactive ,, T-cells and hepatosplenic ,, T-cell lymphomas, they display an important morphological heterogeneity. Moreover, these nonhepatosplenic ,, T-cell lymphomas are essentially not that different from their ,, T-cell receptor for antigen (TCR)-expressing counterparts and thus may be incorporated in the established T-cell lymphoma subclasses. However, subtle differences regarding their histopathological appearance as well as their biological behaviour indicate that further studies to determine the exact significance of TCR expression are required. Such inquiries may contribute to the general understanding of T-cell lymphomagenesis in general, which is still obscure. [source] Unique vascular tumor primary arising in the liver and exhibiting histopathological features consistent with so-called polymorphous hemangioendotheliomaPATHOLOGY INTERNATIONAL, Issue 12 2009Lorenzo Cobianchi Reported herein is an unusual vascular tumor primary arising in the liver and exhibiting unique histopathological features. A 47-year-old woman underwent left hepatectomy because of a large hepatic mass. On histology the tumor had a composite pattern, consisting of angiomatous, retiform and solid areas, formed by oval to cuboidal to spindle cells, that expressed only endothelial markers (CD31 and factor VIII-related antigen). These findings led to the diagnosis of a low-grade vascular neoplasm with morphological features consistent with so-called polymorphous hemangioendothelioma. The tumor was completely resected. At 24 month follow up the patient was alive, without evidence of disease. Polymorphous hemangioendothelioma is a rare vascular neoplasm, with borderline malignant potential, which usually occurs in lymph nodes and, rarely, at extranodal sites. Its classification as an entity has been questioned recently. The unusual morphological features of the present case, which do not fit neatly with any other recognized hemangioendothelioma subtype, indicate that the family of vascular tumors is broader than currently accepted. In addition the present case widens the spectrum of primary vascular tumors arising in the liver. [source] Anaplastic large cell lymphoma in leukemic phase: Extraordinarily high white blood cell countPATHOLOGY INTERNATIONAL, Issue 5 2009Jacqueline T. Nguyen Anaplastic large cell lymphoma (ALCL) is a distinct type of T/null-cell non-Hodgkin lymphoma that commonly involves nodal and extranodal sites. The World Health Organization of lymphoid neoplasms recognizes two types: anaplastic lymphoma kinase (ALK) positive or ALK negative, the former as a result of abnormalities involving the ALK gene at chromosome 2p23. Patients with ALCL rarely develop a leukemic phase of disease, either at the time of initial presentation or during the clinical course. Described herein is a patient with ALK+ ALCL, small cell variant, associated with the t(2;5)(p23;q35), who initially presented with leukemic involvement and an extraordinarily high leukocyte count of 529 × 109/L, which subsequently peaked at 587 × 109/L. Despite chemotherapy the patient died 2˝ months after diagnosis. In the literature review 20 well-documented cases are identified of ALCL in leukemic phase reported previously, with a WBC ranging from 15 to 151 × 109/L. Leukemic phase of ALCL occurs almost exclusively in patients with ALK+ ALCL, most often associated with the small cell variant and the t(2;5)(p23;q35), similar to the present case. Patients with leukemic phase ALK+ ALCL appear to have a poorer prognosis than most patients with ALK+ ALCL. [source] Primary breast non-Hodgkin's lymphoma: A large single center study of initial characteristics, natural history, and prognostic factors,AMERICAN JOURNAL OF HEMATOLOGY, Issue 3 2009Patricia Validire The aims of this study were to define the initial pathological and clinical characteristics, and prognostic factors of patients with primary breast malignant lymphoma (PBL). All patients treated at the Institut Curie for lymphoma with breast involvement were reviewed. A pathological review of all cases was performed. Forty-five cases were selected in whom 38 cases were of diffuse large B-cell lymphoma. A complete analysis was then performed on these 38 patients. Twenty out of 28 cases (71%) of cases were Bcl-2 positive and four out of 28 (14%) had a CD10 positive staining. Peculiar initial characteristics showed nodal involvement in 58% of the cases and two or more extra-nodal sites in 31% of the cases. Among the 37 patients for whom all data were available, and according to the International Prognostic Index, 19 patients (51%) were classified in the low-risk group, 5 cases (14%) in the low- to intermediate-risk group, 6 patients (16%) in the intermediate- to high-risk group, and 7 (19%) case in the high-risk group. At the end of initial therapy, 34 patients (89%) achieved CR. With a median follow-up of 96 months, 18 patients (47%) relapsed of whom 3 had a relapse in central nervous system site. The 5-year disease-free (DFS) and overall survivals (OS) were 54% and 61%, respectively. In multivariate analysis, the presence of 2 or more extranodal sites was prognostic for lower DFS (P = 0.0008) and OS (P = 0.09), and a performance status ,1 was prognostic for lower OS (P = 0.005). Finally, when our series was compared with a historical series of 111 patients with aggressive nodal lymphomas, we observed significant lower survival rates in localized PBL (P < 0.03). Initial breast localization has a pejorative impact on the outcome of patients with Non-Hodgkin's Lymphoma (NHL), with an impressive adverse influence of additional extranodal sites. These results suggest a specific management of NHL with breast involvement. Am. J. Hematol., 2009. © 2008 Wiley-Liss, Inc. [source] Current and possible future treatment of ocular adnexal lymphomasACTA OPHTHALMOLOGICA, Issue 2008A PETTITT Purpose To review the current and possible future therapies of ocular adnexal lymphomas. Methods Ocular adnexal lymphomas represent approx. 8% of all extranodal lymphomas. The majority of these can be classified as extranodal marginal zone (MALT) lymphomas, and are usually staged as Stage IE disease. Results Recommended therapy in Stage IE tumours is low-dose radiotherapy, while disseminated disease (>Stage IIE) is treated with chemotherapy. Although often responding to initial therapy, the MALT lymphomas tend to recur in distant extranodal sites. Few biomarkers are available to aid prediction of either recurrence or systemic dissemination, which occurs in up to 25% of patients. The ocular morbidity associated with current therapies is not insignificant, and, therefore, more effective treatment is being sought. Conclusion The newer treatment options, including rituximab and doxycyclin, will be discussed [source] | ||||||||||||||||||||||