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Background/aims: To validate the prognostic performance of the American Joint Committee on Cancer (AJCC) eighth edition classification for ocular adnexal lymphoma (OAL)., Methods: We performed a retrospective review of 140 consecutive patients treated for primary OAL between March 2010 and September 2017. Associations between T/N/M categories at presentation and disease-related outcomes, including relapse, progression-free survival (PFS) and overall survival (OS) were evaluated., Results: Seventy-nine women and 61 men (median age, 52 (range 20-84) years; median follow-up, 57 (range 7-131) months) were included. Histological subtypes included mucosa-associated lymphoid tissue lymphoma (92.1%, n=129), diffuse large B-cell lymphoma (5.0%, n=7), follicular lymphoma (1.4%, n=2) and mantle cell lymphoma (1.4%, n=2). Patients with ≥T2 disease had significantly higher risks of overall relapse (unadjusted HR)=4.32, p=0.016), decreased PFS (uHR=5.19, p=0.004) and decreased OS (uHR=9.21, p=0.047). Patients with ≥N1 disease had significantly higher risks of overall relapse (uHR=9.17, p<0.001) and decreased PFS (uHR=9.24, p<0.001). M1 disease was significantly associated with higher risks of overall relapse (uHR=3.62, p=0.036), decreased PFS (uHR=5.13, p=0.001) and decreased OS (uHR=9.24, p=0.013). On considering TNM categories as continuous data, the uHRs for per level increase in T, N and M categories were 1.77, 1.83 and 2.30 for overall relapse and 1.72, 1.87 and 2.78 for decreased PFS, respectively (p<0.05 for each comparison)., Conclusion: The T, N and M categories of the AJCC eighth edition classification have prognostic value for relapse and survival among patients with primary OAL. Particularly, nodal/metastatic involvement at presentation indicated less favourable outcome., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Non-follicular small cell lymphomas include several entities whose clinical and pathological descriptions have been refined in the last 20 years. MALT lymphoma, developed at the expense of lymphoid tissue associated with the mucosa, is usually localized to a given organ, but can also disseminate. Some patients with MALT lymphoma can be treated by eradicating the associated infectious agent, whereas local treatment should be preferred for other cases ; disseminated forms and relapsed patients are eligible for anti-CD20 antibodies associated with cytotoxic agents. Patients with mantle cell lymphoma have benefited from many advances, including the use of cytarabine and bendamustine, anti-CD20 antibodies, intensive treatments (autograft) and recently targeted therapy (ibrutinib, inhibitor or the Bruton tyrosine kinase). Patients with splenic nodal marginal zone lymphomas should be evaluated for different options, of which immunochemotherapy remains important. For all these entities, the implementation of treatments may be delayed by several years for certain groups of patients. Although considered as incurable, the prognosis of these pathologies has improved significantly and the majority of patients will be able to live for many years with often treatment-free intervals., (Copyright © 2019. Published by Elsevier Masson SAS.)

Objectives: To evaluate the clinical utility of immune receptor translocation-associated protein 1 (IRTA1) and myeloid nuclear differentiation antigen (MNDA) expression in the diagnosis and classification of marginal zone lymphomas (MZLs)., Methods: IRTA1 was examined using a novel RNA in situ hybridization assay and MNDA expression determined by immunohistochemistry in 127 small B-cell neoplasms, including 80 cases of MZL., Results: IRTA1 expression was detected in 31 (42%) of 74 MZLs vs one (2%) of 43 other small B-cell neoplasms (P < .001). MNDA staining was positive in 51 (64%) of 79 MZLs vs 21 (45%) of 46 non-MZLs (P = .06). MNDA expression was particularly uncommon in follicular lymphoma (3/14, 21%; P = .003 vs MZL). There was no association between MNDA and IRTA1 expression and the presence of monocytoid cytology. IRTA1 expression was less frequent in cases with a diffuse growth pattern., Conclusions: IRTA1 and MNDA are useful markers in the differential diagnosis of MZLs.

Disease Overview: Approximately one-fourth of cutaneous lymphomas are B-cell derived and are generally classified into three distinct subgroups: primary cutaneous follicle center lymphoma (PCFCL), primary cutaneous marginal zone lymphoma (PCMZL), and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT)., Diagnosis: Diagnosis and disease classification are based on histologic review and immunohistochemical staining of an appropriate skin biopsy. Pathologic review and an appropriate staging evaluation are necessary to distinguish primary cutaneous B-cell lymphomas from systemic B-cell lymphomas with secondary skin involvement., Risk Stratification: Disease histology remains the most important prognostic determinant. Both PCFCL and PCMZL are indolent lymphomas that infrequently disseminate to extracutaneous sites and are associated with 5-year survival rates that exceed 95%. In contrast, PCDLBCL, LT is an aggressive lymphoma with an inferior prognosis., Risk-Adapted Therapy: PCFCL and PCMZL patients with solitary or relatively few skin lesions may be affectively managed with local radiation therapy. While single-agent rituximab may be employed for patients with more widespread skin involvement, multiagent chemotherapy is rarely appropriate. In contrast, management of patients with PCDLBCL, LT is comparable to the management of patients with systemic DLBCL., (© 2018 Wiley Periodicals, Inc.)

Post-transplant lymphoproliferative disorders (PTLD) are a known risk for both solid organ transplant and stem cell transplant recipients. Overall transplant recipients have a six fold increase in risk for developing any kind of non-Hodgkin lymphoma and PTLDs occur in up to 10% of SOT recipients. Several new entities have been accepted or renamed in the 2018 update of the WHO classification of tumors of hematopoietic and lymphoid neoplasms, including florid follicular hyperplasia and extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue (MALT-lymphoma) (excluding common locations such as stomach and salivary gland). Other more rare types of PTLD have been reclassified including EBV-positive mucocutaneous ulcer, which is now a recognized diagnosis in its own right and should not be considered polymorphous PTLD. In this paper newly recognized PTLD entities and more unusual PTLDs will be examined., (Copyright © 2018 Elsevier Inc. All rights reserved.)

To explore the distribution of lymphoid neoplasms in Northwest China, the clinical and pathological data of lymphoma patients from 2006 to 2014 were analyzed according to the WHO classification in Xijing Hospital. Of the 3244 cases, mature B-cell neoplasms occupied 60.7%, while mature T/NK-cell neoplasms and Hodgkin's lymphomas (HL) occupied 26.2% and 8.1%, respectively. The most common subtype of lymphoma was diffuse large B-cell lymphoma (35.0%), followed by extranodal NK/T-cell lymphoma, nasal type (ENKTCL) (12.9%) and marginal zone B-cell lymphoma (7.8%). Mixed cellularity (34.0%) was the most common subtype of HL. The especially high proportion of ENKTCL was the most outstanding feature of our study in comparison to previous reports. The mean age of all lymphoid neoplasms cases was 51years and most subtypes showed male predominance, with an average male-female ratio of 1.6. Extranodal lymphomas took up about 60% of all cases and gastrointestinal tract was the most frequently involved site. In conclusion, the distribution of lymphoid neoplasms of Northwest China showed some features similar to previous reports of China and other countries, but some subtypes presented distinct features., (Copyright © 2017. Published by Elsevier Inc.)

Purpose of Review: The purpose of the study is to summarize the current conundrums in the management of marginal zone lymphomas (MZL)., Recent Findings: In 2017, the US Food and Drug Administration (FDA) approved ibrutinib, a first in class Bruton Tyrosine Kinase inhibitor, for the treatment of relapsed/refractory MZL based on pivotal open-label phase II trial demonstrating an overall response rates of 48%. Clinical trials design utilizing chemotherapy-free regimens for relapsed/refractory disease are gaining popularity. Recent studies have identified multiple genetic biomarkers that helped characterize and prognosticate different subtypes of MZL. MZLs are heterogeneous, mostly indolent, malignancies derived from B lymphocytes. Three disease subtypes are recognized, extranodal, nodal, and splenic. The disease characteristics, clinical picture, and treatment algorithms vary considerably based on subtype and site of involvement. Recent discoveries have enhanced our knowledge of the pathogenesis of MZLs leading to development of more accurate prognostic models as well as novel targeted systemic therapies.

Nodal marginal zone B cell lymphomas (NMZLs) are a rare group of lymphoid disorders part of the spectrum of marginal zone B-cell lymphomas, which encompass splenic marginal one B-cell lymphoma (SMZL) and extra nodal marginal zone of B-cell lymphoma (EMZL), often of MALT-type. Two clinicopathological forms of NMZL are recognized: adult-type and pediatric-type, respectively. NMZLs show overlapping features with other types of MZ, but distinctive features as well. In this review, we will focus on the salient distinguishing features of NMZL mostly under morphological/immunophenotypical/molecular perspectives in views of the recent acquisitions and forthcoming updated 2016 WHO classification of lymphoid malignancies., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Lymphomas infiltrating the mediastinum are a challenge for the treating physician as well as for the pathological diagnostics. The clinical scenario is often an emergency situation, while the pathologist is usually confronted only with small biopsy samples. Classical Hodgkin's lymphoma is by far the most frequently occurring lymphoma in the mediastinum and predominantly the nodular sclerosis subtype. In small and very sclerotic specimens it can be difficult to morphologically detect Hodgkin and Reed-Sternberg cells and to identify the characteristic phenotype by immunohistochemistry. Primary mediastinal large B‑cell lymphomas should be distinguished from classical Hodgkin's lymphomas as the treatment is different. This is characterized by the detection of sheets of blast cells, which immunohistochemically show a strong B‑cell phenotype (positivity for CD20 and CD79a), while CD30 can also often be expressed. The intimate biological relationship between classical Hodgkin's lymphomas and mediastinal large B‑cell lymphomas is illustrated by the existence of B‑cell lymphomas with intermediate features (so-called mediastinal grey zone lymphomas). It is important to recognize and diagnose these lymphomas as they are associated with a slightly inferior prognosis. Extranodal thymic marginal zone lymphomas of the mucosa-associated lymphoid tissue (MALT) type are a rare form of lymphoma encountered in the mediastinum, which can be associated with autoimmune diseases. T‑lymphoblastic lymphomas and leukemia, which occur predominantly in children and young adults, represent a rapidly growing precursor cell neoplasia and must be distinguished from thymomas in the differential diagnostics as well as from normal and hyperplastic thymus glands.

The diagnosis of primary cutaneous B-cell lymphoma (CBCL) requires that the search for a more widespread lymphoma has been negative. The clinical presentation, outlook, and treatment options of the common types of CBCLs, with emphasis on differences or similarities to their nodal counterparts, are discussed. Treatment may range from observation to topical therapies to systemic therapies, depending on the histology, degree and area of skin involvement, patient performance, and comorbidities. Rare lymphomas, such as intravascular large B-cell lymphoma and Epstein-Barr virus-positive cutaneous lymphoproliferations that are associated with immunodeficiency, are also briefly described., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to determine the clinicopathological findings and prognosis of small-sized anterior mediastinal tumors (SSAMTs)., Methods: A retrospective study was conducted on 43 patients who underwent surgery between January 1989 and December 2011 for SSAMTs., Results: From the preoperative radiological findings, the tumors were classified into solid (n = 28) and cystic lesions (n = 15). The pathological diagnoses of the solid lesions included thymoma (n = 24), thymic carcinoma (n = 1), mucosa-associated lymphoid tissue lymphoma (n = 1), teratoma (n = 1) and neurofibroma (n = 1), and those of the cystic lesions included thymic cysts (n = 8), thymoma (n = 3), bronchogenic cysts (n = 2), teratoma, (n = 1) and a pericardial cyst (n = 1). The 27 thymomas were composed of stages I (n = 22), II (n = 3), III (n = 1) and IVb (n = 1). The overall survival in the 43 patients was 97.1 % at 5 years. In the 28 patients with solid lesions, the overall survival was 95.8 % at 5 years. All patients with cystic lesions were still alive at the last follow-up., Conclusion: Cystic lesions of SSAMTs were benign lesions or stage I thymoma, and most of the solid lesions of SSAMTs were stage I or II thymomas. SSAMTs are good candidates for video-assisted thoracic surgery procedures, as conversion to sternotomy can be selected based on the intraoperative findings of pericardial invasion and a rapid pathological diagnosis of thymic carcinoma.

The biological and clinical significance of a clonal B-cell lymphocytosis with an immunophenotype consistent with marginal-zone origin (CBL-MZ) is poorly understood. We retrospectively evaluated 102 such cases with no clinical evidence to suggest a concurrent MZ lymphoma. Immunophenotyping revealed a clonal B-cell population with Matutes score ≤2 in all cases; 19/102 were weakly CD5 positive and all 35 cases tested expressed CD49d. Bone marrow biopsy exhibited mostly mixed patterns of small B-lymphocytic infiltration. A total of 48/66 (72.7%) cases had an abnormal karyotype. Immunogenetics revealed overusage of the IGHV4-34 gene and somatic hypermutation in 71/79 (89.8%) IGHV-IGHD-IGHJ gene rearrangements. With a median follow-up of 5 years, 85 cases remain stable (group A), whereas 17 cases (group B) progressed, of whom 15 developed splenomegaly. The clonal B-cell count, degree of marrow infiltration, immunophenotypic, or immunogenetic findings at diagnosis did not distinguish between the 2 groups. However, deletions of chromosome 7q were confined to group A and complex karyotypes were more frequent in group B. Although CBL-MZ may antedate SMZL/SLLU, most cases remain stable over time. These cases, not readily classifiable within the World Heath Organization classification, raise the possibility that CBL-MZ should be considered as a new provisional entity within the spectrum of clonal MZ disorders.

Bone marrow involvement at the time of diagnosis is uncommon in patients with primary cutaneous marginal zone B-cell lymphoma (PCMZL). Moreover, in these patients such involvement is rarely found in isolation on diagnosis. Typically the few patients with PCMZL who have early bone marrow involvement also present secondary nodal or visceral involvement, which is detected by other staging studies (usually computed tomography). In recent years, this has given rise to some debate about whether a bone marrow biopsy should be routinely performed in patients diagnosed with PCMZL in view of the good prognosis and low incidence of bone marrow infiltration and/or extracutaneous involvement in this type of lymphoma., (Copyright © 2012 Elsevier España, S.L. and AEDV. All rights reserved.)

Primary cutaneous B-cell lymphomas (PCBCLs) are defined as lymphomas with a B-cell phenotype that present in the skin without evidence of systemic or extracutaneous disease at initial presentation, after adequate staging. In non-Hodgkin lymphomas, the skin is the second most common site of extranodal involvement after the gastrointestinal tract. PCBCLs are histologically very similar to their nodal counterparts, and these histologic similarities can lead to confusion about both therapy and prognosis. This article will summarize the clinical, pathologic, and diagnostic features of the 3 main types of PCBCL: primary cutaneous follicle center lymphoma, primary cutaneous marginal zone lymphoma, and primary cutaneous diffuse large B-cell lymphoma, leg-type, and the appropriate evaluation and staging procedures for each of these entities., (Copyright © 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Concepts on cutaneous mucosa-associated lymphoid tissue lymphomas have been one of the most evolving areas of interest in dermatopathology in the past few decades. Researchers not only have modified the classification of such entities from the old concept of immunocytoma but have also developed a better understanding about how such lymphomas evolve from a chronic stimulating environment and about their associated genetic alterations. Because the latest advances point to the existence of several types of cutaneous mucosa-associated lymphoid tissue lymphomas, we believe that clarity about the concepts on marginal-zone cells can only contribute to the understanding of this fascinating type of lymphoma.

To clarify the relationships between marginal zone lymphomas (MZLs) and Waldenström macroglobulinemia/lymphoplasmacytic lymphomas (WM/LPLs), immunoglobulin heavy chain variable gene (IGHV) features were analyzed and the occurrence of MYD88 L265P mutations was identified in a series of 123 patients: 53 MZLs from the spleen (SMZLs), 11 from lymph nodes (NMZLs), 28 mucosa-associated lymphatic tissue (MALT) lymphomas and 31 WM/LPLs. SMZLs were characterized by overrepresentation of IGHV1-2 gene rearrangements with a canonical motif, without selection pressure and with long CDR3 segments. NMZLs had increased frequencies of IGHV3 genes. The IGHV gene was unmutated in most cases, often with long CDR3 segments. MALT lymphomas were usually associated with a mutated IGHV gene, but with the absence of selection pressure. WM/LPLs were associated with an IGHV3-23 overrepresentation and high IGHV mutation rate, with features of selection pressure and short CDR3 segments. MYD88 L265P mutations were almost restricted exclusively to WM/LPL patients. Taken all diagnoses together, all patients with MYD88 L265P mutations had an immunoglobulin M peak and almost all patients except one had bone marrow infiltration. These results demonstrate that the history of antigen exposure of the four entities studied was different and MYD88 L265P was specifically associated with WM/LPLs. WM/LPL may thus be functionally associated with constitutive nuclear factor-κB activation.

Splenic marginal zone lymphoma (SMZL) with or without villous lymphocytes is a low-grade malignant lymphoma, which primarily involves the spleen, the bone marrow and the blood. Even though the prognosis of the disease is good and there is a long overall survival, about two thirds of the patients will require treatment and in 10% of the patients the lymphoma will transform into a high-grade malignant lymphoma. The treatment of SMZL remains empirical, since there is lack of large, prospective studies. Without such studies, the best treatment modality cannot be established.

Recent epidemiology studies have identified a steady increase in the incidence of cutaneous lymphomas over the past few decades. Although possible explanations for this increased incidence include heightened awareness of these conditions as well as a more refined diagnostic acuity by dermatologists and pathologists, an increase secondary to environmental factors cannot be discounted. Our understanding of cutaneous lymphomas keeps evolving. Consequently, our knowledge and understanding of cutaneous lymphomas requires reconsideration of past dogma and critical revision of the new proposals. In this article, some hot topics and important new findings in the field are reviewed., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Aims: Diffuse large B cell lymphoma (DLBCL) occasionally shows an interfollicular pattern of proliferation (DLBCL-IF) preserving lymphoid follicles. In this study, clinicopathological findings in 31 cases of DLBCL-IF were analysed., Methods and Results: The study group comprised 20 males and 11 females, with ages ranging from 41 to 87 (median 69) years. The primary site was lymph node in 25 cases, and unknown in six due to advanced stage at diagnosis. Eight cases were clinical Stage I, 10 were Stage II, four Stage III, and nine Stage IV. A polymorphous pattern of proliferation containing large B cells and inflammatory cells was found in about 60% of cases. The overall survival rate of the DLBCL-IF patients was better than that of a DLBCL control group (log-rank test; P < 0.05). Multivariate analysis revealed that an interfollicular pattern of proliferation showed marginal significance for favourable prognosis (P = 0.069). Immunohistochemical double staining with antibodies for HLA-DR/CD68 (markers for M1-tumour-associated macrophage [M1-TAM]) or CD163/CD68 (M2-TAM) revealed that all DLBCL-IF patients with a low M2 count were alive at the end of observation., Conclusions: These findings suggest that DLBCL-IF is a clinicopathological entity distinct from ordinary DLBCL. The possible origin of tumour cells in DLBCL-IF from marginal zone B cells is discussed., (© 2012 Blackwell Publishing Ltd.)

Purpose: The aim of this study was to compare endoscopic macroscopic classification with fluorine-18 fluorodeoxyglucose (F-18 FDG) uptake in gastric mucosa-associated lymphoid tissue (MALT) lymphoma and to investigate the usefulness of F-18 FDG positron emission tomography (PET) for diagnosing gastric MALT lymphoma., Materials and Methods: Sixteen patients with gastric MALT lymphoma who underwent F-18 FDG PET and gastrointestinal imaging modalities were included in this study. Sixteen healthy asymptomatic participants undergoing both F-18 FDG PET and endoscopy for cancer screening were in the control group. We investigated the difference of F-18 FDG uptake between the gastric MALT lymphoma and the control group and compared the uptake pattern in gastric MALT lymphoma with our macroscopic classification., Results: The endoscopic findings of 16 gastric MALT lymphoma patients were classified macroscopically as chronic gastritis-like tumors (n = 6), depressed tumors (n = 5), and protruding tumors (n = 5). Abnormal gastric F-18 FDG uptake was observed in 63% of tumors in the gastric MALT lymphoma group and 50% of cases in the control group. The median maximum standardized uptake values for gastric MALT lymphoma patients and control group were 4.0 and 2.6, respectively, the difference of which was statistically significant (P = 0.003). F-18 FDG uptake results were positive for all protruding tumors but only 50% for chronic gastritis-like tumors and 40% for depressed-type tumors., Conclusions: F-18 FDG PET may be a useful method for evaluating protrusion-type gastric MALT lymphoma. When strong focal or diffuse F-18 FDG uptake is detected in the stomach, endoscopic biopsy should be performed, even if the endoscopic finding is chronic gastritis.

Background and Aim: The incidence of extra nodal non Hodgkin lymphoma (ENL) is rising throughout the world. However, data regarding ENL as a group is limited. The aim was to study the epidemiological and histomorphological trends of primary ENL (pENL) in India., Material and Methods: The biopsy materials from sixty eight patients with pENL (45 male, 23 female, M:F= 1.9:1), diagnosed over a five year period (2005-2009), were analysed and pathologically reclassified according to the World Health Organization (WHO) classification, 2008 criteria., Results: Primary extra nodal non Hodgkin lymphomas constituted 22.0% (68/308) of all non Hodgkin lymphomas (NHL). The mean age at presentation for pENL and primary nodal NHL was 43 years and 58 years, respectively with a male predilection (M: F=2:1). Central nervous system (CNS) constituted the most common extranodal site (20/68, 29.5%) followed by gastrointestinal tract (17/68, 25%), and nose/nasopharynx (8/68, 11.8%). Diffuse large B-cell lymphoma (DLBCL, not otherwise specified), extranodal marginal lymphoma of mucosa associated lymphoid tissue (MALT) type, and B cell NHL unclassified (U) were the three most common histological types observed. T-cell phenotype was rarely noted (4%). Follicular lymphomas and anaplastic large cell lymphoma, seen among nodal NHL, were absent at extra nodal sites. Majority (41/68, 60%) of the patients with pENL were immunocompetent and 55% were in stage I-II with favorable prognosis., Conclusion: Central nervous system was the most common site of ENL, followed by gastrointestinal tract. Majority of pENL occurred in immunocompetent hosts with a favorable prognosis.

Background: Marginal zone lymphomas are indolent B-cell lymphomas associated with autoimmunity and chronic inflammation. The two most frequent variants are mucosa associated lymphoid tissues marginal zone lymphomas and splenic marginal zone lymphomas. The aim of the study was to determine if it is possible to classify splenic and gastric lymphomas according to karyometric features., Methods: The material consisted of 16 splenic and 14 gastric lymphomas. The measurements were done with the AnalySIS image analysis system. In each case at least 100 nuclei were selected, and 19 different geometric parameters were measured., Results: On statistical analysis, the nuclei of splenic and gastric lymphomas showed differences in most parameters, but significant overlap of the values was present. Neural networks were trained and used for classification of the data. By this method, the nuclei were properly classified with a sensitivity of 0.75 and specificity of 0.71. In addition, in all the cases the majority of the nuclei were properly classified, thus allowing correct classification of all the cases into "splenic" or "gastric"., Conclusion: These results support the view that mucosa-associated lymphoid tissue lymphomas and splenic marginal-zone lymphomas are separate entities.

Purpose of Review: Indolent B-cell lymphomas that are supposed to derive from marginal zone encompass three distinct entities: extranodal marginal zone lymphoma (MZL) or mucosa-associated lymphatic tissue (MALT), nodal MZL (NMZL) and splenic MZL (SMZL). Although MALT lymphoma is well characterized and extensively studied at the clinical and molecular levels, SMZL and NMZL remain incompletely characterized. However, during the last years, the clinical and molecular heterogeneity of SMZL has been clarified. The recent 2008 WHO classification has maintained the distinction between the three diseases according to the organ where it arises and introduced a new provisional category of unclassified splenic lymphoma for overlapping entities, splenic diffuse red pulp lymphoma (SDRPL) and hairy cell leukemia-variant (HCL-V)., Recent Findings: Recent findings in SMZL contributed to a better characterization, including the few cases associated with hepatitis C, the recurrence of 7q deletion and the possibility of CD5 expression. Furthermore, the peculiar pattern of immunoglobulin heavy chain genes mutations and the biased usage of immunoglobulin heavy chain variable region genes (IGHV)1-2 segment are suggestive of a T-independent antigen driven proliferation, at least at initial steps. This review will focus on recent findings and differential diagnosis with SDRPL and HCL-V., Summary: The conjunction of morphologic, cytogenetic and clinical data has increased diagnosis reproducibility.

Marginal zone B-cell lymphomas (MZLs) have been divided into 3 distinct subtypes (extranodal MZLs of mucosa-associated lymphoid tissue [MALT] type, nodal MZLs, and splenic MZLs). Nevertheless, the relationship between the subtypes is still unclear. We performed a comprehensive analysis of genomic DNA copy number changes in a very large series of MZL cases with the aim of addressing this question. Samples from 218 MZL patients (25 nodal, 57 MALT, 134 splenic, and 2 not better specified MZLs) were analyzed with the Affymetrix Human Mapping 250K SNP arrays, and the data combined with matched gene expression in 33 of 218 cases. MALT lymphoma presented significantly more frequently gains at 3p, 6p, 18p, and del(6q23) (TNFAIP3/A20), whereas splenic MZLs was associated with del(7q31), del(8p). Nodal MZLs did not show statistically significant differences compared with MALT lymphoma while lacking the splenic MZLs-related 7q losses. Gains of 3q and 18q were common to all 3 subtypes. del(8p) was often present together with del(17p) (TP53). Although del(17p) did not determine a worse outcome and del(8p) was only of borderline significance, the presence of both deletions had a highly significant negative impact on the outcome of splenic MZLs.

Background: EUS-guided FNA and Tru-cut biopsy (TCB) is highly accurate in the diagnosis of lymphoma. Subclassification, however, may be difficult in low-grade non-Hodgkin lymphoma and Hodgkin lymphoma., Objective: To determine the yield of EUS-guided biopsy to classify lymphoma based on the World Health Organization classification of tumors of hematopoietic lymphoid tissues., Design: Retrospective study., Setting: Tertiary referral center., Patients: A total of 24 patients referred for EUS-guided biopsy who had a final diagnosis of lymphoma or "highly suspicious for lymphoma.", Interventions: EUS-guided FNA and TCB combined with flow cytometry (FC) analysis. MAIN OUTCOMES MEASUREMENT: Lymphoma subclassification accuracy of EUS guided biopsy., Results: Twenty-four patients were included in this study. Twenty-three patients underwent EUS-FNA, and 1 patient had only TCB. Twenty-two underwent EUS-TCB combined with FNA. EUS correctly diagnosed lymphoma in 19 out of 24 patients (79%), and subclassification was determined in 16 patients (66.6%). Flow cytometry correctly identified B-cell monoclonality in 95% (18 out of 19). In 1 patient diagnosed as having marginal-zone lymphoma by EUS-FNA/FC only, the diagnosis was changed to hairy cell leukemia after a bone marrow biopsy was obtained. EUS had a lower yield in nonlarge B-cell lymphoma (only 9 out of 15 cases [60%]) compared with large B-cell lymphoma (78%; P = .3 [Fisher exact test])., Limitations: Retrospective, small number of patients., Conclusion: EUS-guided biopsy has a lower yield to correctly classify Hodgkin lymphoma and low-grade lymphoma compared with high-grade diffuse large B-cell lymphoma., (Copyright 2010 American Society for Gastrointestinal Endoscopy. Published by Mosby, Inc. All rights reserved.)

Classic splenic marginal zone lymphomas are CD5-, CD10-, CD23-, CD43-, and usually IgD+ with biphasic white pulp nodules. However, the 2008 World Health Organization classification accepts splenic marginal zone lymphomas with monophasic marginal zone-like white pulp nodules and recognizes a group of unclassifiable splenic small B-cell lymphomas. To explore the relationship of classic splenic marginal zone lymphomas to these other less well-defined splenic lymphomas, a multiparameter study of 47 splenic marginal zone lymphomas and unclassifiable splenic small B-cell lymphomas was performed. Seventeen of 31 splenic marginal zone lymphomas were biphasic, and 14 were monophasic (90%-100% marginal zone-like white pulp nodules). Sixteen cases were unclassifiable splenic small B-cell lymphomas, most lacking a marginal zone-type component. There were many clinical similarities between the 3 groups, including similar survivals. Monophasic and unclassifiable cases were less likely to have a typical splenic marginal zone lymphoma phenotype (28.6%, 23.1%) compared with biphasic cases (86.7%), usually because of IgD negativity (P < .003). Thirty-four of 42 (81%) cases had cytogenetic abnormalities by fluorescence in situ hybridization; and 17 of 20 (85%), by classical cytogenetics. The most frequent fluorescence in situ hybridization abnormalities among the splenic marginal zone lymphomas were del(7)(q31) (26%), +12 (25%), and +3q27 (27%); and among the unclassifiable cases, +12 (50%) and +3q27 (36%). Five of 6 unclassifiable cases with exclusively small non-marginal zone-like lymphocytes involving both white and red pulp had +12 compared with 9 of 34 other cases (P < .02). CDK6 (2 cases) and BCL3 (1 case) rearrangements were only seen in the unclassifiable group. These results support including both biphasic and monophasic cases as splenic marginal zone lymphomas, but suggest that the lack of a non-marginal zone-like population in the monophasic group is associated with some biologic differences. They also demonstrate a relatively large proportion of unclassifiable cases, including a group with frequent +12., (Copyright 2010 Elsevier Inc.)

Background: The prognostic values of the International Prognostic Index (IPI) and the Follicular Lymphoma International Prognostic Index (FLIPI) have widely been demonstrated in diffuse large B-cell lymphoma and follicular lymphoma. No attempts to assess their applicability in MALT lymphoma have been made so far., Patients and Methods: A total of 143 patients with MALT-lymphoma were analysed. Parameters of both IPI and FLIPI were retrospectively assessed and correlated with relapse and time to relapse as markers of clinical course., Results: According to IPI, 96 patients (67%) were classified as low, 22 (15%) low-intermediate, 17 (12%) high-intermediate and 8 (6%) as high risk. FLIPI identified 99 patients (70%) at low risk, 35 (24%) at intermediate and 9 (6%) at high risk. After a median follow-up time of 39.5 months, 123 patients were alive and 46 patients had relapsed (median time to relapse 27 months). IPI significantly correlated with time to relapse, with the typical differentiation into low, low-intermediate and high risk groups. FLIPI divided patients into three groups, but the low and intermediate risk groups showed a similar clinical course. In terms of additional progonostic factors, univariate analysis suggested autoimmune disease and multifocal disease as correlated with relapse. Multiple regression analysis, however, identified only extragastric disease as predictive of relapse (p=0.001)., Conclusion: Our data demonstrate that both IPI and FLIPI are able to discriminate prognostic subgroups in patients with MALT-lymphoma. However, the low and intermediate group of the FLIPI did not appear to prognostically differ.

Context: Diagnosis and classification of primary intraocular lymphoma can be challenging because of the sparse cellularity of the vitreous specimens., Objective: To classify and clinically correlate intraocular lymphoma according to the World Health Organization (WHO) classification by using vitrectomy specimens., Design: Clinical history, cytologic preparations, flow cytometry reports, and outcome of 16 patients diagnosed with intraocular lymphoma were reviewed., Results: The study group included 10 women and 6 men. The mean age of the patients was 63 years (range, 19-79 years). Eleven patients had central nervous system involvement and 6 patients had systemic involvement. All cases were adequately diagnosed and classified according to the WHO classification by using combination of cytologic preparations and 4-color flow cytometry with a limited panel of antibodies to CD19, CD20, CD5, CD10, and kappa and lambda light chains. The cases included 9 primary diffuse large B-cell lymphomas of the CNS type; 2 diffuse large B-cell lymphomas, not otherwise specified; 1 extranodal, low-grade, marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT); 1 precursor B-lymphoblastic lymphoma; and 3 peripheral T-cell lymphomas, not otherwise specified. Of note, all 11 cases of diffuse large B-cell lymphoma were CD10-. All the patients received systemic chemotherapy and radiation therapy. Only 4 patients were free of disease at last follow-up (range, 18 months to 8 years), with severe visual loss., Conclusions: Intraocular lymphoma cases can be adequately classified according to the WHO classification. Diffuse large B-cell lymphoma, CD10- and most likely of non-germinal center B-cell-like subgroup, is the most common subtype of non-Hodgkin lymphoma in this site, in contrast to ocular adnexal lymphoma for which MALT lymphoma is the most common subtype.

According to the WHO-EORTC classification of cutaneous lymphomas, primary cutaneous marginal zone B-cell lymphoma are now well characterized. We report here a case of primary cutaneous marginal zone B-cell lymphoma in a 51 year-old man in which the diagnosis was made using both histology and immunopathology. The patient had no remarkable medical history, no history of either acute inflammation or insect bite, and presented with a 5 cm solitary asymptomatic erythematous firm, multinodular and infiltrated plaque on the back for 12 months. Histological examination and immunohistochemical study of a cutaneous biopsy provided a differential diagnosis between B cell lymphoma and lymphocytoma cutis. Full body work up revealed no signs of extracutaneous dissemination. The patient underwent surgical excision of the nodule. Histological examination showed a histological and immunophenotyping profile typical of primary cutaneous marginal zone B-cell lymphoma. The lesion was completely excised with clear margins and no recurrence occurred after a 12 month-follow-up period. Primary cutaneous marginal zone B-cell lymphoma are low-grade lymphomas that have an indolent course and a high tendency to recur. They should be differentiated from lymphocytoma cutis and from the other types of cutaneous B cell lymphomas that have a different course and prognosis.

In the past 50 years, we have witnessed explosive growth in the understanding of normal and neoplastic lymphoid cells. B-cell, T-cell, and natural killer (NK)-cell neoplasms in many respects recapitulate normal stages of lymphoid cell differentiation and function, so that they can be to some extent classified according to the corresponding normal stage. Likewise, the molecular mechanisms involved the pathogenesis of lymphomas and lymphoid leukemias are often based on the physiology of the lymphoid cells, capitalizing on deregulated normal physiology by harnessing the promoters of genes essential for lymphocyte function. The clinical manifestations of lymphomas likewise reflect the normal function of lymphoid cells in vivo. The multiparameter approach to classification adopted by the World Health Organization (WHO) classification has been validated in international studies as being highly reproducible, and enhancing the interpretation of clinical and translational studies. In addition, accurate and precise classification of disease entities facilitates the discovery of the molecular basis of lymphoid neoplasms in the basic science laboratory.

In the World Health Organization classification of tumors of hematopoietic and lymphoid tissues, the group of marginal zone lymphomas comprises three different entities, namely the extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, the nodal marginal zone B-cell, and the splenic marginal zone B-cell lymphoma (with or without circulating villous lymphocytes). This article addresses each of the marginal zone lymphomas.

Splenic marginal zone lymphoma (SMZL), characterized in the WHO classification of lymphoid tumors, is a rare disorder comprising less than 1% of lymphoid neoplasms; only a few series concerning this entity have been published. Although this type of lymphoma is well defined histologically, its histogenesis remains obscure. Moreover, specific biological markers are still lacking and immunophenotype profile is not specific. These and other reasons, such as the existence of cytogenetic subtypes, have led to some authors to suspect that SMZL constitutes a heterogeneous entity. We have analyzed a series of sixteen SMZL cases from four hospitals in our community, from a clinical, biological and pathological point of view. When compared with those reported in the literature, our findings show three main differences: our patients less frequently showed an intrasinusoidal bone marrow infiltration pattern; the presence of a serum monoclonal component was rarely seen; and CD5-positive SMZL cases appear to be more common than previously thought.

Context: Marginal zone lymphomas (MZLs) are the most common lymphomas encountered in the orbit and ocular adnexa. The accurate categorization of these lymphomas is critical to avoid undertreatment or overtreatment., Objective: To identify features of orbital MZLs that distinguish them from other lymphomas and reactive lymphoid infiltrates and support the categorization of orbital MZL as mucosa-associated lymphoid tissue (MALT)-type MZLs., Design: Biopsies from 149 patients with lymphoid lesions of ocular adnexa were examined. Additional immunohistochemical stains and fluorescence in situ hybridization study for the MALT1 locus were performed in selected cases, and patient charts were reviewed., Results: A total of 115 lymphomas and 34 reactive infiltrates were identified, of which B-cell lymphomas constituted 92% and MZLs constituted 54% of all lymphomas. Certain clinical features (young age, race, bilaterality) favored a reactive infiltrate, but none were diagnostic. Histologic features, such as infiltrative lesions, reactive B-cell follicles, and lymphoepithelial lesions, overlapped between reactive infiltrates and conjunctival MZL. In contrast to conjuctival MZL, orbital MZL infrequently showed reactive follicles, rarely showed epithelial tissue, and did not show lymphoepithelial lesions. Cytogenetic abnormality involving the MALT1 locus was demonstrated in only 15% of ocular adnexal MZLs., Conclusion: Many MZLs of orbital soft tissue lack key features associated with MALT-type MZL, and the designation MALT lymphoma should be avoided in their diagnosis.

We compared the morphologic findings of different types of marginal zone B-cell lymphoma (MZL) involving the bone marrow (BM), including 18 splenic (SMZL), 6 extranodal (mucosa-associated lymphoid tissue lymphoma), and 6 nodal cases. The median percentage of BM involvement was 15%, and multiple overlapping patterns of infiltration were observed in all MZL types. The most frequent patterns were nodular (87%) and interstitial (63%). A focal sinusoidal pattern of involvement was found in one third of SMZLs and rarely in MALT lymphoma. Germinal centers (GCs) were uncommon in routinely stained BM biopsy sections and were observed only in SMZL. However, antibodies specific for CD21 and CD23 highlighted follicular dendritic cells in most MZLs of all types. MZLs cannot be distinguished from each other by examining BM sections alone. However, a sinusoidal pattern or presence of GCs is suggestive of SMZL. Furthermore, correlation with the CBC count can further enhance the reliability of diagnosing SMZL.

To clarify the clinical presentation and outcome of nodal marginal zone B-cell lymphoma (NMZBL), 65 Japanese patients with this disease were studied and compared with the published literature from western countries. The clinical findings of our 65 cases were similar to those of their cases in some aspects: (1) 58% of the patients were > 60 years old (median age, 64 years); (2) there was a slight female predominance; (3) 90% of the patients exhibited asymptomatic lymphadenopathy in the head and neck area; (4) only a minority of patients had B symptoms (6%) and poor performance status (8%); and (5) only 5% of patients were positive for M-protein. However, the 65 patients in this series exhibited relatively longer 5-year overall survival (85%) and failure-free survival (60%) than the NMZBL series published in western literature, suggesting that NMZBL should be classified as indolent lymphoma. Moreover, based on the histological findings, we further classified four histological subtypes as follows: (1) splenic type (n = 7); (2) floral type (n = 9); (3) mucosa-associated lymphoid tissue (MALT) type (n = 29); and (4) diffuse large B-cell lymphoma (DLBCL) + MALT type (n = 20). DLBCL + MALT type exhibited significantly poorer 5-year overall survival than the splenic variant. The recognition of DLBCL + MALT type appears important. No API2-MALT1 fusion transcript was detected in any of the 14 cases examined.

B-cell clonality, assessed by PCR for amplification of the VDJ region of the immunoglobulin heavy chain gene (IgH), is used to support the diagnosis of gastric mucosa-associated lymphoid tissue (MALT) lymphoma (GML). It has also been described in simple gastritis cases, without any histological hint for lymphoma, especially in the presence of lymphoid follicles. We analyzed a randomly selected series of 130 gastric biopsies with histologically described lymphoid follicle formation and investigated these for the prevalence of B-cell clonality using different PCR-based methods to discuss its usefulness in the differential diagnosis of GML. A seminested PCR for the IgH gene was performed and evaluated by agarose gel electrophoresis, GeneScan technique, and melting-curve analysis. The majority of cases revealed histologically chronic active Helicobacter pylori gastritis. Monoclonality was detected in 7.5% (10 of 130) and 7% (9 of 130) of samples using GeneScan technique and melting-curve analysis, respectively. In eight of eight samples investigated, monoclonality was not demonstrated in deeper sections of the same biopsy using GeneScan technique, favoring the diagnosis of a reactive process rather than overt lymphoma. Electrophoresis proved more difficult to interpret and revealed clonal cases in 14% (18 of 130). We conclude that GeneScan technique and melting curve analysis are the methods of choice for clonality analysis in gastric biopsies. Analyses of different deep sections with advanced PCR technology might be the method of choice for future analyses. In our opinion, the question of whether detected monoclonality can be interpreted as malignant lymphoma is still open.

The purpose of this study was to document the frequency and distribution of karyotypic changes present at diagnosis in 103 non-MALT marginal zone cell lymphoma (MZL) patients. This cytogenetic analysis of a large cohort extends previous observations and allows the identification of new cytogenetic features. Abnormalities identified in more than 15% of patients included +3/+3q (37%), 7q deletions (31%), +18/+18q (28%), 6q deletions (19%), +12/+12q (15%) and 8p deletions (15%). Trisomy 3/3q, 7q deletions, +18 and +12 were seen in different combinations in more than 30% of patients in comparison to 2% in lymphocytic lymphomas/chronic lymphocytic leukemias, 1% in mantle cell lymphomas and 7% in follicular lymphomas. The marked propensity of these abnormalities to be recurrently associated with the same tumoral clone of individual karyotypes allowed the delineation of a cytogenetic profile that may help to distinguish non-MALT MZL among other mature B-cell neoplasms. If +3/3q, +12/+12q, and 6q, 7q and 8p deletions were significantly associated with clinical prognostic factors previously reported to influence survival and time to progression, patients displaying these abnormalities did not experience a significantly shorter time to progression.

Primary colon lymphomas are very uncommon gastrointestinal tumors, more so in the descending colon. We would like to present this clinical entity with the case of a 33-year-old woman in order to inform them about the case of Primary colon lymphoma that was encountered and to review the literature. In her case, the diagnosis was suspected from the clinical symptoms, images and fiber endoscopy study. The diagnosis was confirmed by anatomopathology and inmunohistoquimic study. The woman was subjected to exploratory laparotomy and the final diagnosis was given to be high-grade MALT type Lymphoma B cells (Non-Hodgkins Lymphoma Big Cell) big cells) EII2.

Marginal-zone lymphoma (MZL) includes three subtypes depending on the site of lymphoma involvement: extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT-lymphoma); splenic MZL; and nodal MZL. Beside a common cell-of-origin and similarities concerning a possible chronic antigenic stimulation by microbial pathogens and/or autoantigens, the clinical presentation is very different with symptoms related to lymphoma location. MALT and splenic MZL present with an indolent disease with good performance status, no B symptoms, and no adverse prognostic factors and are associated with long survival. Patients with nodal MZL present with a more aggressive disease and have a shorter failure-free survival. Clinical and biological prognostic factors identified in reported series are heterogeneous. The optimal treatment has yet to be defined for the three subtypes, and current strategies will be described in this review.

Background: The classification of mucosa associated lymphoid tissue (MALT) lymphoma is based on characteristic morphologic and immunophenotypic patterns, with distinctive chromosomal aberrations. The critical first step is diagnosis on evaluating H&E-stained sections. We performed an inter-observer study to determine the degree of agreement among pathologists in evaluating gastric lymphocytic infiltrates for MALT lymphoma., Methods: A set of 41 H&E-stained gastric sections (36 endoscopic biopsies and 5 surgically resected sections) that ranged from simple gastritis to primary gastric lymphoma was reviewed separately and independently by 17 participants including hematopathologists, pathologists with a special interest in gastrointestinal pathology, and general pathologists. The participants were from the United States, Europe, and Japan. Results were entered into a standardized data collection form and the results were analyzed using kappa statistics. Monte Carlo simulation was used to adjust for multiple biases., Results: Overall, interobserver reproducibility in the morphologic evaluation of gastric MALT was suboptimal. The kappa statistic was 0.3 for simple gastritis, low-grade MALT and for high grade MALT lymphoma. Monte Carlo simulation suggested that the degree of disagreement was directly related to the pathologist's experience in evaluating gastric biopsies for MALT lesions. However, after conjointly reviewing all cases, the Houston workshop agreed on findings that would increase the reproducibility of diagnosis, especially for pathologists with limited experience with this disease. These included the availability of macroscopic data, extensive sampling, the presence of lymphoepithelial lesions, immunophenotyping and particularly abnormal mucosa localization of B-cells, in addition to other molecular finding such as monoclonality and translocation t (11;18). The group also agreed on the need for standardizing the terminology currently used to facilitate future comparison between studies., Conclusions: Though the study shows poor agreement on morphologic MALT lymphoma categorization, the Houston workshop suggested recommendations that should increase the diagnostic accuracy and reproducibility of MALT lymphoma diagnosis. A follow up workshop will be organized to measure the diagnostic reproducibility for MALT lymphoma using the suggested recommendations.

Gastric MALT lymphoma shows unique features including regression by Helicobacter pylori eradication and API2-MALT1 fusion. We performed a molecular and clinicopathologic study for 115 cases. All eradication-responsive cases were devoid of API2-MALT1 fusion. All tumors positive for the fusion and all negative for H. pylori infection were nonresponsive to the eradication. Consequently, gastric MALT lymphomas were divided into three groups: Eradication-responsive and fusion-negative (group A, n = 72), eradication-nonresponsive and fusion-negative (group B, n = 22), and eradication-nonresponsive and fusion-positive (group C, n = 21). Group A tumors were characterized by low clinical stage and superficial gastric wall involvement, and group C tumors by low H. pylori infection rate, advanced clinical stage, and nuclear BCL10 expression. All group C tumors showed exclusively low-grade histology. Group B tumors, which have not been well recognized, frequently showed nodal involvement, deep gastric wall involvement, and advanced clinical stage, and sometimes an increased large cell component. A multivariate discriminant analysis revealed that responsiveness to the eradication could be predicted accurately by negative API2-MALT1 fusion, positive H. pylori infection, low clinical stage, and superficial gastric wall invasion, the former being the most important factor for the prediction. This 3-group categorization may be helpful for a comprehensive understanding of gastric MALT lymphoma.

Although several recurrent genetic aberrations are known to occur in MALT lymphoma, no comprehensive study on the most prevalent MALT lymphoma-associated genetic aberrations is available. We therefore screened 252 primary MALT lymphomas for translocations t(11;18)(q21;q21), t(14;18)(q32;q21), and t(1;14)(p22;q32), and trisomies 3 and 18. The above-listed translocations occurred mutually exclusively and were detected overall in 13.5, 10.8, and 1.6% of the cases; trisomy 3 and/or 18 occurred in 42.1%. The frequency at which the translocations occurred varied markedly with the primary site of disease. The t(11;18)(q21;q21) was mainly detected in pulmonary and gastric tumors, whereas the t(14;18)(q32;q21) was most commonly found in lesions of the ocular adnexa/orbit, skin, and salivary glands. Trisomies 3 and 18 each occurred most frequently in intestinal and salivary gland MALT lymphomas. Our results demonstrate that the three translocations and trisomies 3 and 18 occur at markedly variable frequencies in MALT lymphoma of different sites.

Introduction: Primary Gastric Lymphoma is an uncommon malignancy among gastric malignancies. Histology of the Primary Gastric Lymphoma is varied and the extranodal marginal zone B-cells lymphoma is specially significant on account of its potential remission with antibiotic therapy., Objectives: Observe the clinical characteristics of patients with Primary Gastric Lymphoma, assess the most relevant endoscopic findings, identify the factors that influence survival and evaluate the effects of therapy., Materials and Methods: The study is an observational, analytical, cross evaluation including 169 patients with histological diagnosis of Gastric Lymphoma, treated at the National Institute for Neoplastic Diseases, Lima, Peru, from January 1995 to December 2000. Staging was based on the Ann Arbor system, modified by Musshoff and histology, on the REAL-WHO classification. The statistical analysis included the student-t and the chi-square tests. Survival data were entered using the Kaplan Meier curves and prognosis factors, using the Cox regression test., Results: The sample represents patients from the Peruvian Coast, with a mean age of 55 years old and slight predominance of female patients (54.4%). Signs and symptoms are unspecific. Clinical stage I-II corresponds to 75% of the patients. The endoscopic pattern of multiple ulcerated lesions is characteristic of the Gastric Lymphoma. A total of 71% of the patients with extranodal marginal zone B-cells lymphoma showed total remission of the disease with antibiotic therapy (5/7). The histological type of the Gastric Lymphoma in the 169 patients was as follows: Large, diffuse, B-cells Lymphoma, 137 patients, extranodal marginal zone B-cells lymphoma, 16 patients, peripheral T-cell Lymphoma, 6 patients, anaplastic large T-cell Lymphoma, 3 patients, undetermined Lymphoma, 3 patients, mantle cell Lymphoma, 2 patients, adult T-cell Lymphoma, 1 patient and follicular Lymphoma, 1 patient. Global survival after 36 months was of 61.34%, survival according to the histological type was of 92.31% for extranodal marginal zone B-cells Lymphomas, 62.21% for large, diffuse B-cells Lymphomas and 29.63% for T-cell Lymphomas. Survival after 36 months in patients in clinical stage I-II treated with chemotherapy, was of 82.16%, with surgery, 71.89% and with surgery and chemotherapy, 70.39, with similar results in all three groups (p: 0.6530). The groups classified according to the international index, showed a clear difference between them (p:0.0000). The univariate analysis revealed that Zubrod (p:0.0000) DHL (p:0.0073) disease remission (p:0.0000) stage (p:0.0000) treatment (p:0.0000) and location (p:0.0000) had statistical significance. Multivariate analysis showed that in the Cox regression model, remission (OR:13,342, p:0.0000) and location (OR:2.375, p:0.041) fall within the equation of such function., Conclusions: The multiple ulcerated lesions are characteristic of the Gastric Lymphoma. Remission of the disease in the extranodal marginal zone B-cells Lymphoma is evidenced with the use of antibiotic therapy (5/7). Chemotherapy in patients with EC I-II achieves survival results similar to those treated with surgery and with a combination of both. Validity of the international index is confirmed and the multivariate analysis proved that remission and location of the disease have statistical significance.