Extranodal Presentation Research Articles

Either Combined-Modality Or Radiotherapy Alone Provide Favorable Outcome In Stage I-II Mantle Cell Lymphoma: A Report Of 82 Patients From The International Lymphoma Radiation Oncology Group (ILROG)

Patients with Mantle cell lymphoma (MCL) often present with advanced-stage and have a poor prognosis. Although MCL is highly radiosensitive, RT is rarely used other than for palliation. Stage I-II presentation is rare, and there is only limited data on treatment outcome with less aggressive combined modality approaches or RT alone. To assess the outcome of this group of patients, we retrospectively reviewed 82 patients referred to radiation oncology at 9 ILROG institutions between 1990 and 2012. Of the 82 patients, 65 (79%) were males, 44 (54%) were > 60 years; 47 (57%) were stage I, 61 (74%) presented in the head and neck areas, 51 (62%) had extranodal presentation, 14 (17%) were bulky (>5 cm). Treatment approaches consisted of combined modality (CM) with chemotherapy and consolidative radiation therapy (RT) in 66 (80%) and definitive RT alone in 16 (20%). The median radiation dose used was 35 Gy (range 12-45). Chemotherapy consisted of cyclophosphamide, adriamycin, vincristine, and prednisone (CHOP) or CHOP-like, regimens in 62 (57%), rituximab was added to chemotherapy in 51 (62%). The median follow-up was 60 months (range 5-245). At last follow up, 26 (32%) patients relapsed (70% of relapses were outside the radiation field) and 21 (26%) died (2 deaths unrelated to disease or treatment).The 5-and 10-years overall survivals (OS) were 71% and 63%, respectively. The 5-and 10-years disease free survivals (DFS) were 75% and 39%, respectively.On univariate analysis, the following characteristics were significantly associated with DFS and OS: age as a continuous variable (HR=1.05, p=0.005 for DFS; HR=1.05, p=0.006 for OS) and bulky disease (HR=2.78, p=0.03 for DFS; HR= 3.18, p=0.02 for OS). On multivariate analysis, both DFS and OS were significantly influenced by: age as a continuous variable (HR=2.09, p=0.03; HR=1.05, p=0.005), bulky disease (HR=2.23, p=0.05; HR=3.1, p=0.03), and female gender only affected DFS (HR=2.16, p=0.03). ConclusionPatients who presented with stage I-II MCL and were treated with programs that included consolidative local radiotherapy or even radiotherapy alone demonstrated favorable disease control and relatively long survival. It appears that radiotherapy as a sole modality can offer in selected patients a comparable DFS and OS to a combined modality approach. Disclosures:No relevant conflicts of interest to declare.SurvivalAll (%)Combined modality (%)RT alone up-front(%)5-years OS71689210-years OS6362745-years DFS75738910-years DFS393657

Epstein–Barr virus is rarely associated with diffuse large B cell lymphoma in Taiwan and carries a trend for a shorter median survival time

AimsEpstein–Barr virus (EBV)-positive diffuse large B cell lymphoma (DLBCL) of the elderly is characterised by frequent extranodal involvement, a morphological spectrum from polymorphous to monomorphous and a poor prognosis. The...

Unusual case of metachronous EBV‐associated B‐cell and NK/T‐cell lymphoma mimicking polymyositis‐diagnostic challenges and pitfalls

Unusual case of metachronous EBV‐associated B‐cell and NK/T‐cell lymphoma mimicking polymyositis‐diagnostic challenges and pitfalls

Adult T cell leukaemia/lymphoma in Kerala, South India: are we staring at the tip of the iceberg?

This study comes from Regional Cancer Centre, Trivandrum, in the state of Kerala, South India. Regional Cancer Centre is a comprehensive cancer centre catering to the population of the State of Kerala and the adjoining states of Tamil Nadu and Karnataka. This study is an analysis of leukaemic phase, nodal and extranodal presentations of adult T cell leukaemia/lymphoma (ATLL) in our institute. This study aims to document the alarming incidence of ATLL in this part of South India. This study includes 32 cases, which were diagnosed as compatible with ATLL over a period of 26 months.Our diagnostic approach to a typical case starts with a detailed history, clinical examination with emphasis on skin lesions,evaluation of laboratory findings especially seurm calcium and lactate dehydrogenase values. A peripheral blood examination would be followed by ancillary tests like flow cytometric immunophenotyping of peripheral blood or histopathological examination with immunostaining of submitted tissue biopsy. The last step is the confirmatory serology test for serum human T cell lymphotropic virus type 1 (HTLV-1) antibody. We report for the first time from the state of Kerala, South India, 15 patients with HTLV-1-positive ATLL. This high incidence of ATLL in our state population hitherto not reported is worrisome and entails routine screening for serum HTLV-1 before invasive procedures and among voluntary blood donors in our population.

Trends in incidence, therapy and outcome of localized nodal and extranodal marginal zone lymphomas: declining incidence and inferior outcome for gastrointestinal sites

Population-based series analyzing clinical implications of nodal versus extranodal presentation of marginal zone lymphoma (MZL) are lacking. We studied clinical differences and trends in incidence, therapy and survival of nodal and extranodal MZL, and of MZL at different extranodal sites, in a population-based cohort. All patients with localized (Ann Arbor stage I and II) nodal (n = 211), splenic (n = 54) and extranodal (n = 1449) MZL, diagnosed between 1994 and 2010, were selected from The Netherlands Cancer Registry. Between 1994 and 2010 the incidence of nodal and extranodal MZL increased. The incidence of gastric MZL decreased. Patients with nodal MZL received more chemotherapy and targeted therapies than their extranodal counterparts. A trend in time toward less chemotherapy and more irradiation was observed. Overall survival (OS) curves for nodal and extranodal MZL overlapped (5-year OS 76% and 77%, respectively). Patients with a primary gastrointestinal (GI) localization had inferior OS compared to patients with non-GI extranodal MZL (5-year OS 71% and 85%, p < 0.0001). Patients with localized extranodal MZL presented more commonly with stage I disease, but their clinical presentation and survival were otherwise similar to patients with localized nodal MZL. MZL arising in the GI tract does not have a good prognosis and requires a different treatment approach.

Lymphoproliferative disorder that resembles heptosplenic lymphoma during maintenance treatment for T‐cell acute lymphoblastic leukemia

A 6-year-old male presented with a testicular mass, hepatosplenomegaly, and a pleural effusion while undergoing maintenance chemotherapy for treatment of T-cell acute lymphoblastic leukemia (T-ALL). He was subsequently diagnosed with a lymphoproliferative disorder that resembled hepatosplenic lymphoma (HSL). While the extranodal presentation and the protracted yet aggressive clinical course are consistent with HSL, the findings of monosomy 8 and polymorphic cell populations are unique and have not been previously described in this type of lymphoma.

Pediatric precursor B-cell lymphoblastic lymphoma presenting with extensive skeletal lesions

Lymphoblastic lymphoma (LL) of the B cell type is uncommon, and extranodal presentation is even rarer. It is difficult to suspect this diagnosis without clinically obvious lymph nodal mass or bone marrow involvement. A 3-year-old girl presented with progressive pain and swelling of the right knee joint of 3 months duration. Radiograph revealed expansile lytic lesion at the supracondylar area of the right femur; with pathological fracture and multiple lytic areas in both femora. She neither had lymphadenopathy nor organomegaly. Her blood counts, peripheral smear examination and bone marrow examination were normal. Right supracondylar biopsy revealed diagnosis of a precursor B cell LL. Computerized tomography scan revealed a hypodense, poorly enhancing lesion in the left adnexal region. Although rare, precursor B-cell LL may present with extensive bone lesions. Early and accurate diagnosis of this entity is very important due to its high cure rates.

Undertreatment of patients with localized extranodal compared with nodal diffuse large B-cell lymphoma

Population-based studies analyzing clinical implications of nodal versus extranodal (EN) presentation of diffuse large B-cell lymphoma (DLBCL) are scarce. We studied clinical differences and trends in incidence, treatment and survival of nodal and EN DLBCL in a population-based cohort. All patients newly diagnosed with localized (Ann Arbor stage [AAS] I and II) nodal (n = 5124) and EN (n = 4776) DLBCL, and primary mediastinal B-cell lymphoma (PMBL; n = 88), diagnosed between 1989 and 2010, were selected from the Netherlands Cancer Registry. Primary EN disease was correlated with older age and more favorable clinical stage (AAS I). The age standardized incidence rates for men with localized EN DLBCL, and for men and women with localized PMBL, increased significantly, whereas the age standardized incidence rates of all other subgroups remained stable. The stomach was the most common EN localization. Patients with EN disease received less chemotherapy and targeted therapy than their nodal counterparts, irrespective of age and period of diagnosis. Their 5-year overall survival (OS) was 48% vs. 54% in the nodal group, but in multivariate analysis primary extranodal presentation was not independently associated with inferior survival. This population-based study shows clinically relevant differences between localized nodal and EN DLBCL and PMBL. Since patients with EN were significantly less often optimally treated, we advocate better interaction between medical disciplines.

Flow cytometry CD45‐negative B‐NHL: A case report of a diffuse large B‐cell lymphoma without extranodal involvement

The loss of CD45, the leukocyte-common antigen, has been described in rare cases of large B-cell lymphoma (LBCL) subtypes with extranodal involvement by immunohistochemical methods. Here we report a case of a patient with LBCL, with no extranodal lesions, which is CD45 negative by flow cytometry (FC) immunophenotyping. Immunophenotyping and DNA content analysis was performed by multiparametric FC on lymph node and bone marrow aspirate obtained from a 65 year old male patient. Malignant B-lymphocytes were CD5-, CD10+/++, CD11c-, CD19+, CD20+/++, CD23-, CD34-, CD38-/+, CD45-, CD79b++/+++, BCL2 overexpressed, FMC7++, IgM++/+++, TdT- with Lambda light chain restriction. This pathological cellular population showed near-diploid DNA content, with a high proliferate rate. To our knowledge, we describe the first case of a CD19+ B-cell non-Hodgkin lymphoma without expression of CD45 detected by FC, and the first case without extranodal involvement presentation. This case is reported not only because it is a rare one but also to raise awareness of FC users of its correct diagnosis.

Cutaneous Rosai-Dorfman Disease Recurrence in Infraorbital Region

Rosai-Dorfman disease (RDD), also named sinus histiocytosis with massive lymphadenopathy, is a rare, idiopathic, and benign disorder that classically presents as a painless massive bilateral cervical lymphadenopathy. In cases of extranodal presentations, such as cutaneous RDD, it may not involve the classic manifestation. The diagnosis is usually made by histopathologic analysis. Surgery is suggested in the cases of significant cosmetic deformity or when there is fatal or functional obstruction. We reported a case of extranodal RDD that recurred in the infraorbital region postoperatively.

Clinicopathologic Features of B-Cell Lineage Neoplasms With Aberrant Expression of CD3

CD3 expression by immunohistochemistry was historically considered restricted to T-lineage or NK-lineage neoplasms but recently has been reported in rare cases of mature B-cell neoplasms, frequently in association with Epstein-Barr virus. Here, we describe the pathologic features of 21 B-cell lineage neoplasms that express CD3 protein by immunohistochemistry: 12 diffuse large B-cell lymphomas (DLBCLs); 2 plasmablastic lymphomas (PBLs); 4 plasma cell neoplasms; 2 Burkitt lymphomas; and 1 nodal follicular lymphoma, grade 3A. CD20 expression was negative or only partially positive in 13/21 cases. Epstein-Barr virus was positive in 3/20 tested cases (2 PBLs and 1 DLBCL). All tested neoplasms (14/14) had clonal immunoglobulin gene rearrangements, and no clonal T-cell gene rearrangements were detected (0/14). The 12 DLBCLs segregated into 2 main groups: 7 demonstrated features of plasmacytic differentiation but did not meet criteria for PBL, and 5 had anaplastic features. In addition to morphology, other features shared among the DLBCLs with plasmacytic differentiation, the plasma cell neoplasms, and the PBLs included extranodal presentation, cytoplasmic localization of CD3, and lack of expression of other T-cell antigens in most cases. In contrast, DLBCLs with anaplastic features and the single follicular lymphoma coexpressed multiple T-cell antigens in a predominantly membranous pattern and presented with nodal disease in a relatively younger patient population. Our data expand the spectrum of morphologic, phenotypic, and clinical features of B-cell neoplasms aberrantly expressing CD3. As these neoplasms often lack typical expression of B-cell antigens, knowledge of these features will help avoid misclassification.

Primary pulmonary extranodal natural killer/T-cell lymphoma: nasal type with multiple nodules

To the Editors: Primary pulmonary lymphoma (PPL) is an uncommon disease representing only 3–4% of extranodal non-Hodgkin’s lymphoma (NHL), and <1% of NHL [1]. The true incidence of PPL other than NHL is unknown, while primary pulmonary T-cell lymphoma is extremely rare. Natural killer (NK)/T-cell lymphoma usually shows an extranodal presentation. Nasal NK/T-cell lymphomas occur in the nose and the upper aerodigestive tract. Extranasal NK/T-cell lymphomas represent the counterpart of nasal NK/T-cell lymphomas and can involve every other part of the body. Primary sites of involvement are mainly the skin, soft tissue, gastrointestinal tract and testis [2]. Although some cases of primary pulmonary T-cell lymphoma have been reported, there are only two reports written in English to date [3, 4]. Here, we describe a case of primary pulmonary NK/T-cell lymphoma with multiple nodules in both lung fields. The patient was a 50-yr-old Japanese male with an abnormal shadow on chest radiography in a health examination (fig. 1a). He was an office worker with no history of dust inhalation or asbestos exposure and was a current smoker (50 pack-yrs). He had a history of hypertension and gallstones. He had a high fever of over 39°C for 10 days before the health examination, and had appetite loss and general fatigue. A chest computed tomography (CT) on admission revealed multiple nodules, measuring a maximum of 40 mm in size, were in both lung fields (fig. 1b–d). Administration of broad-spectrum antibiotics did not resolve his symptoms. Bronchoscopic examination and CT-guided needle biopsy did not give a definite diagnosis. Laboratory data showed elevation of lactate dehydrogenase, transaminases, hepatobiliary enzymes and an inflammatory reaction without cytopenia. Two sets of blood …

The role of hematopoietic SCT in adult Burkitt lymphoma

Adult Burkitt lymphoma (BL) is an aggressive disease characterized by frequent extranodal presentation, bulky disease and a rapid clinical course. Although intensive chemotherapeutic regimes result in long-term disease-free survival in most patients, a significant proportion of patients will have high-risk disease that may be refractory or that will relapse. In these patients, the role of hematopoietic SCT is not well defined, especially in the era of modern chemoimmunotherapy. Upfront auto-SCT has been reported to be feasible in patients who have high-risk features at presentation, and in whom it is a clinical option. In patients with relapsed disease, auto-SCT can result in a PFS of 30-40%. Allo-SCT is an option in relapsing patients with a sibling or matched related donor who may not be eligible for, or may have previously received, an auto-SCT; the role of RIC and T-cell depletion is not well defined. Disease status at transplant is the most significant predictor of outcome in patients undergoing SCT. Here we review the available evidence pertaining to SCT in patients with BL, including in those who are HIV positive (HIV+) and those with B-cell lymphoma unclassified (BCLU). Prospective studies in the era of modern intensive chemoimmunotherapeutic regimes are required to delineate the precise role of transplantation for BL. Developments in molecular diagnostics, incorporation of FDG-PET and minimal residual disease monitoring along with new therapies may further assist in refining treatment algorithms.

Primary Spinal Natural Killer/T-Cell Lymphoma Successfully Treated With Concurrent Chemoradiotherapy and Autologous Stem Cell Transplantation

This case report shows primary natural killer (NK)/T-cell lymphoma originating in the spine as the first case of successful treatment with autologous stem cell transplantation, along with case presentation and literature review. For the proper treatment of this spinal tumor, the pathologic confirmation includes immunophenotypic profiling, the understanding of tumor characteristics, and the consideration of spinal stability are necessary. NK/T-cell lymphomas are rare, aggressive neoplastic disorders with a distinctive clinical pathologic profile and an unfavorable clinical prognosis. The extranodal nasal-type NK/T-cell lymphomas, which are characterized by an extranodal presentation and an aggressive clinical course, are prevalent in Asia and Central and South America. A 49-year-old woman presented with thoracic back pain that developed 3 months before she was admitted to Chung-Ang University. The radiological image showed a 6 × 4 × 5-cm mass lesion within the T6-T7 vertebral body. The histopathology was consistent with the finding of NK/T-cell lymphoma. Treatment with chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisone) in combination with radiotherapy (total dose, 30 Gy) was done, and salvage therapy with autologous stem cell transplantation was performed. Two-year follow-up magnetic resonance image showed shrinking remnant mass without spinal deformation and/or instability. Rebiopsy for remnant mass, nasal area study, and positron emission tomographic scan were performed to determine the tumor recurrence. There was no evidence of recurrence at the 2-year follow-up evaluations. Some primary NK/T-cell lymphomas that originate at sites other than the nasal cavity/nasopharynx do not present with typical clinical features and symptoms. A primary NK/T-cell lymphoma of the spine should be considered in the differential diagnosis of primary bone tumors. We report the first case of successful treatment with chemoradiotherapy and autologous stem cell transplantation in a patient with a primary spinal NK/T-cell lymphoma.

Non-Hodgkin lymphoma in Chile: a review of 207 consecutive adult cases by a panel of five expert hematopathologists

The distribution of subtypes of non-Hodgkin lymphoma (NHL) in Latin America is not well known. This Chilean study included 207 consecutive cases of NHL diagnosed at five cancer centers in the capital, Santiago, and one center in Viña del Mar. All cases were reviewed and classified independently by five expert hematopathologists according to the 2001 World Health Organization classification of NHL. A consensus diagnosis of NHL was reached in 195 of the 207 cases (94%). B-cell lymphomas constituted 88% of NHL, and diffuse large B-cell lymphoma (DLBCL, 38.5%) and follicular lymphoma (25.1%) were the most common subtypes. There was a high frequency of marginal zone B-cell lymphoma (10.3%), as well as of extranodal natural killer (NK)/T-cell lymphoma, nasal type (2.6%) and adult T-cell leukemia/lymphoma (0.5%). Extranodal presentation was seen in 74 of the 195 cases (38%) and the most common extranodal presentation was in the stomach (37.6%). The most common gastric lymphoma was DLBCL (54.5%) followed by mucosa-associated lymphoid tissue (MALT) lymphoma (41%). Overall, the frequency of NHL subtypes in Chile is between that reported in Western and Eastern countries, which is probably a reflection of the admixture of ethnicities as well as the environment and socioeconomic status of its population.

Flow cytometric detection and quantification of CD56 (neural cell adhesion molecule, NCAM) expression in diffuse large B cell lymphomas and review of the literature

To report unusual CD56 (neural cell adhesion molecule, NCAM) expression on diffuse large B cell lymphoma (DLBCL). CD56 expression was first detected and quantified on tissues obtained from five cases of DLBCL by flow cytometry (FC), then confirmed by immunohistochemistry. The CD56 expression pattern was heterogeneous among the cases [the molecular equivalent of soluble fluorochrome (MESF) level ranged from 2214 to 133 466]. All were CD10 and Bcl-6 positive, suggesting their germinal centre origin; one was also CD5 positive. An extranodal presentation occurred in three of five cases. CD56 expression in B cell lymphoma is a rare occurrence. FC is able to identify aberrant immunophenotypes that can be useful in the identification and monitoring of B cell lymphoma subtypes. The presence of CD56 reported by the literature on certain DLBCL with extranodal presentation might be related to mechanisms involved in growth and expansion.

A case of Rosai-Dorfman Disease in a pediatric patient with cardiac involvement

Rosai-Dorfman disease (RDD) involves abnormal proliferations of oddly behaving histocytes that are not derived from the Langerhan's Cell linage. These collections tend to occur within lymph nodes, with occasional extra nodal presentation. While RDD is a rare entity itself, extra nodal cases are even more so, with even fewer reporting cardiac involvement, and previously only in adults. This report describes the disease in a pediatric patient who had the unique feature of an extra nodal cardiac mass. The patient, who was known to have sickle cell disease, was initially erroneously thought to have acute chest syndrome. Sudden changes in the patient's status, including development of 3rd degree heart block, demanded investigation with additional imaging. Chest CT revealed a mass arising from the cardiac interatrial septum and encircling the entire thoracic aorta. Imaging features of Rosai-Dorfman disease are nonspecific, complicating the diagnosis. We present this case with discussion of this extremely uncommon entity. We describe the diagnostic methods, the differential diagnosis, and the treatment options.

Histiocytic Sarcoma of the Esophagus

Purpose: Introduction: Histiocytic sarcomas are rare hematolymphoid neoplasms, with frequent extranodal manifestations. Several reports of GI involvement, including the liver, spleen, stomach and colon, have been detailed in the literature. Due to its non-specific morphology, immunophenotypic studies are vital for diagnosis. We present a case of primary esophageal invasion by histiocytic sarcoma, which has not been documented to date. Case: A 46 year old male patient presented with abdominal pain, dysphagia and weight loss over 6 months. Examination revealed epigastric tenderness and non-tender hepatomegaly. Profound leukocytosis was noted, and progressive escalation of all his liver function tests. A CT scan of the abdomen revealed a 6 cm mass at the gastroesophageal junction, multiple hypodense hepatic lesions consistent with metastases and extensive intraabdominal lymphadenopathy. A 10 cm, ulcerative, necrotizing mass just above the gastroesophageal junction was discovered on endoscopy, and biopsy was reported as a histiocytic cell neoplasm. Immunophenotyping revealed positive CD68, Vimentin and EMA. Staining for myeloperoxidase, CD1a, CD30, CD34 and CD117 were absent. Based on these findings, a diagnosis of histiocytic sarcoma was established. Unfortunately, the patient developed intercurrent sepsis with rapid deterioration, dying from multi-organ failure. Discussion: Histiocytic sarcoma is an extremely rare and aggressive malignancy of monocyte/macrophage origin. The median age group affected is the 5th decade, and extranodal presentation is common. Various sites of gastrointestinal involvement have been documented, presenting with hepatosplenomegaly, abdominal pain, intestinal obstruction, bleeding and perforation. This case illustrates a hitherto unreported manifestation, with primary involvement of the esophagus. The diagnostic criteria has been very controversial and recently refined based on immunohistochemical markers. This demonstrates consistent expression of CD68, with CD45 and S100 being equivocal. Markers for myeloid cells (MPO) and Langerhan's cells (CD1a) are conspicuously negative. Resection is favored for localized tumors, and systemic chemotherapy otherwise. Recently, a case treated with stem cell transplantation has been described.Figure: No Caption available.

Primary breast lymphoma and HIV/AIDS

Primary extranodal lymphoma of the breast is rare and accounts for 0.04 - 0.53% of all malignant tumours, less than 1% of non-Hodgkin’s lymphomas (NHLs) and 1.7% of extranodal NHLs.1 Extranodal lymphoma arises from tissue other than lymph nodes and sites that normally contain no lymphoid tissue. This case describes the clinical and treatment dilemma posed by extranodal presentation in an AIDS-NHL patient.

Clinicopathological features of Epstein-Barr virus-positive diffuse large B-cell lymphoma in elderly

To investigate the clinicopathological features of EB virus positive diffuse large B-cell lymphomas (EBV + DLBCL) of the elderly. Four hundred and ninety-six cases of DLBCLs were retrospectively studied by in situ hybridization (ISH) to detect the EBV in tumor cells, and by immunohistochemistry to evaluate the expression of CD10, CD20, CD30, CD79a, bcl-6, bcl-2, MUM-1, CD5, CD3, TIA-1 and Ki-67 protein. Their clinicopathological correlations were analyzed. Of the 59 cases of EBV + DLBCL, 48 cases were EBV positive. The median age of these EBV + DLBCLs was 73 years with male predominance (1.4:1). There were 11 cases with nodal presentation only, 18 cases with extra-nodal presentation and 19 cases with both lymph nodal and extra-nodal involvements, whereas about one third cases with more than one extra-nodal involvement. Thirty-five patients presented with advanced disease (Ann Arbor stage III/IV). A performance status was available in 36 cases and 5 cases had performance status of more than 1. Seven of 30 patients were found with high lactate dehydrogenase value (more than twice of the normal). An IPI-score was calculated in 30 cases and 18 cases had an intermediate/high IPI-score (3-5). The median survival for these patients was 35 months. Morphologically, EBV + DLBCLs of the elderly generally showed a diffuse and polymorphic proliferation of large lymphoid cells with varying degrees of reactive components including small lymphocytes, plasma cells, histiocytes, and epithelioid cells. These tumor cells were frequently characterized by a broad range of B-cell maturation, containing centroblasts, immunoblasts, and Hodgkin- and Reed-Sternberg (HRS)-like giant cells. The study cohort was further morphologically divided into large cell lymphoma subtypes (n = 33) and polymorphic lymphoma subtypes (n = 14) and one case with mixed subtype. Immunohistochemical studies showed that tumor cells were positive for CD20 (47/48) and/or CD79a (45/45) in almost cases. Tumor cells were MUM-1-positive in the majority of the cases (44/47) and were stained for CD10 or bcl-6 in a few cases. Expression of bcl-2 and CD30 was observed in 80.0% (28/35) and 28.9% (11/38) cases, respectively, and most of the cases (33/39) had a high proliferative index (by Ki-67 with a 50% cut-off point). Compared with other EBV + DLBCLs, except the older age and low frequency of bcl-6 staining, no other significant differences were observed in EBV + DLBCLs of the elderly. EBV + DLBCLs of the elderly constitute a distinct clinicopathologic subtype of DLBCL, although many clinical and histological features with EBV + lymphomas are similar with that of younger ages. Differential diagnosis from other types of lymphomas should also be considered.