Gastric T-cell Lymphoma Research Articles

The epidemiology and prognosis of patients with primary gastric T-cell lymphoma in the SEER program.

Primary gastric T-cell lymphoma (PG-TCL) is a rare hematological malignancy with few data reported. The objective of this study is to investigate the epidemiology, clinical characteristics, and survivals of PG-TCL. Totally, 164 patients with PG-TCL from 1975 to 2016 extracted from the Surveillance, Epidemiology, and End Results Program (SEER) database were analyzed. Kaplan-Meier method was applied to plot overall survival (OS) and cancer-specific survival (CSS). The prognostic factors of OS and CSS were explored by Cox proportional hazard regression. Nomograms were constructed to predict survival possibilities. The age-adjusted incidence rate of PG-TCL was 0.0091 per 100,000 person-years and increased with age. The median age at onset was 65 years old with male predominance. The major histological type was peripheral T-cell lymphoma, NOS (63.4%). The 1-, 2-, and 5-year OS were 45.5%, 34.7%, and 23.5%, respectively while the 1-, 2-, and 5-year CSS were 47.4%, 37.3%, and 29.6%, respectively. Multivariate Cox analysis demonstrated that age at diagnosis, use of chemotherapy, and radiotherapy were the independent prognostic factors for OS. Chemotherapy combined with radiotherapy could significantly improve patients' OS compared with chemotherapy alone. Moreover, age at diagnosis and use of chemotherapy were also the independent prognostic factors for CSS. Nomograms for PG-TCL were developed to predict 1-, 2-, and 5-year OS possibilities. The predictability of nomograms was verified by high concordance index and good agreement with the predicted value in calibration plots. PG-TCL is a rare neoplasm with low incidence. Patients with PG-TCL generally exhibited poor prognosis. Use of chemotherapy plus radiotherapy was associated with favorable OS.

Novel gammaherpesvirus associated with primary gastric T-cell lymphoma in a free-ranging giant armadillo in Brazil.

The number of viral-associated neoplasms reported in wildlife has increased over the last decades, likely because of growing research efforts and a potentially greater burden of carcinogenic pathogens. Herein, we describe a primary gastric T-cell lymphoma in one free-ranging giant armadillo (Priodontes maximus) from Brazilian Pantanal infected by a novel gammaherpesvirus, proposed as Cingulatid gammaherpesvirus 1 (CiHV-1). By chromogenic in situ hybridisation against Epstein-Barr virus some neoplastic cells were labeled. Subsequently, a molecular screening was carried out to detect the occurrence of this pathogen in other giant armadillos in the same region. Overall, this novel virus was detected in 14.3% (3/21) of the tested giant armadillos. We suggest this herpesvirus, the first in Xenarthra, as a plausible aetiology of the neoplasm. The implications of CiHV-1 for this species are uncertain; while no outbreaks of disease have been recorded, the present study raises concerns. Further research is warranted to assess the real significance of CiHV-1 and its potential oncogenic role in this species.

Endoscopic and pathologic motifs for the clinical diagnosis of Epstein-Barr virus-associated gastric cancer.

ObjectivesBased on the recent therapeutic trends for gastric cancer (GC), the clinical impact of the diagnosis of Epstein–Barr virus (EBV)‐associated GC (EBVaGC) appears to be important. We retrospectively analyzed endoscopic and pathologic motifs of GC lesions to narrow the number of candidates for EBV testing.MethodsWe performed EBV tests for 32 upper gastrointestinal lesions of 32 patients in the clinical setting. These tests were ordered by endoscopists or by pathologists without an endoscopist's order. EBV‐encoded small RNA1 (EBER1) in situ hybridization was used for the EBV tests. The endoscopic motif for the EBV test was the location in the upper part of the stomach or remnant stomach, mainly the depressed type with a submucosal tumor‐like protrusion of the lesion. The pathologic motif was carcinoma with lymphoid stroma (CLS) or CLS‐like histology of the lesion. We retrospectively analyzed the results of EBV tests for the endoscopic and pathologic motifs.ResultsThe final pathological diagnoses of the 32 subjects were 26 GCs including CLS, gastric endocrine cell carcinoma, gastric hepatoid carcinoma, gastric T‐cell lymphoma, gastritis of the remnant stomach, esophageal adenocarcinoma, and esophageal squamous cell carcinoma. When nontypical lesions were excluded, the EBER1‐positive rate was 42.3% (11/26) in GCs. Of the 14 GC lesions ordered examined by endoscopists, three (21.4%) were EBVaGC. Eight of the 12 (66.7%) GCs ordered examined by pathologists were EBVaGC.ConclusionsThe pathologic motif is expected to be useful and the endoscopic motif may be helpful for EBVaGC diagnosis.

Mycosis fungoides and gastric T-cell lymphoma: A case report.

Mycosis fungoides (MF) is a cutaneous malignant lymphoma with an extended clinical course. MF presents in series of dermatological manifestations, beginning with patches and plaques of the skin, and eventually evolving into tumours. Often MF can occur for extended periods without worsening of external symptoms, while the disease advances internally in organs such as lymph nodes, liver, spleen, lung, bone marrow, gastrointestinal tract, pancreas and kidney. The present report presents a clinical case in which gastrointestinal symptomatology occurred a decade after the first dermatological manifestation. Immunohistochemical analysis of the skin, along with small bowel biopsies revealed evidence of gastric T-cell lymphoma. To the best of our knowledge, the present study is the first to describe such a case in the literature.

Remarkably long overall survival of a 15-month-old Australian shepherd dog with gastric T-cell lymphoma

A 15-month-old female intact Australian shepherd dog was presented with a history of acute, frequent vomiting and progressive lethargy. Only a painful abdomen was detected on physical examination. Laboratory results...

CD20-positive primary gastric T-cell lymphoma poorly responding to initial treatment with rituximab plus CHOP, and a literature review

There have been rare reported cases of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) that co-expressed CD20. A 44-year-old Japanese male was initially misdiagnosed as CD20-positive diffuse large B-cell lymphoma with a background of reactive CD3-positive T-cells in the stomach. After four cycles of R-CHOP [rituximab plus cyclophosphamide (CY), doxorubicin, vincristine, and prednisolone (PSL)], total gastrectomy with regional lymph node dissection was performed due to the poor response to R-CHOP. A final diagnosis of CD20-positive primary gastric PTCL-NOS was made based on the immunohistochemical, flow cytometric, and molecular genetic findings. In the present case, CD20 immunostaining for T-cell lymphoma cells in tumor tissue varied; in a large part, these were strong to weak-positive, and in some parts, absent. We additionally reviewed the literature focusing on CD20-positive PTCL-NOS treated with rituximab. The administration of rituximab has been performed as an initial treatment in 11 cases, including the case reported here. The response was good in cases with high expression of CD20, while it was poor in cases with variable intensity in CD20 staining, which is consistent with our experience in the present case. The efficacy of rituximab may be associated with intensity of CD20 expression in T cells and its homogeneity in the tumor tissue.

Clinicopathologic study of 7 cases of gastric T-cell lymphoma

To study the clinicopathologic characteristics of gastric T-cell lymphoma. The clinicopathologic features of 7 cases of gastric T-cell lymphoma were retrospectively reviewed. Immunohistochemical study, T-cell receptor gene rearrangement analysis and evaluation of Epstein Barr virus (EBV) status were also performed. The median age at onset of gastric T-cell lymphoma was 45 years. The male-to-female ratio was 6 to 1. The clinical information was available in 6 cases; and one of them had history of persistent diarrhea and 5 had hypoproteinemia. Histologically, 5 cases consisted of large lymphoma cells and the remaining 2 cases showed mainly medium-sized cells. Intraepithelial lymphoma cell infiltration was found in one case. The lymphoma cells of all cases were negative for CD20 and CD79a. CD3 and TIA-1 expression was noted in 6 of the 7 cases. CD5, βF-1 and CD30 were positive in 4 cases and CD4 was positive in 3 cases. In-situ hybridization for Epstein-Barr virus-encoded RNA was negative. Clonal T-cell receptor gene rearrangement was demonstrated in all cases. Gastric T-cell lymphoma is a rare type of malignant lymphoma, with distinctive clinicopathologic characteristics.

Synchronous Colonic and Gastric Peripheral T-cell Lymphoma in an Elderly Patient

Synchronous Colonic and Gastric Peripheral T-cell Lymphoma in an Elderly Patient

Clinicopathological characteristics of primary gastric T‐cell lymphoma

To investigate the clinicopathological characteristics of 20 primary gastric T-cell lymphoma (GTCL) cases without human T-lymphotropic virus type I infection in Japan, a non-endemic area for coeliac disease. Fifteen cases had no history of persistent diarrhoea or severe hypoproteinaemia. Histologically, 13 cases (65%) consisted of large cell lymphoma and seven (35%) were of medium-sized cells. Intraepithelial lymphoma cell invasion was found in three cases (15%). Two of 10 surgical cases (20%) showed intramucosal tumour cell spreading with enteropathy-like features. Helicobacter pylori CagA gene was detected in three of 10 cases (30%). The lymphoma cells of all 20 cases were positive for CD3 and/or TCRbetaF1 and negative for CD56. CD4- and CD8- lymphoma was found in 11 cases (55%), CD4+ lymphoma in seven (35%) and CD8+ lymphoma in two (10%). CD30+, CD5+ and CD25+ lymphomas were detected in nine (45%), 10 (50%) and 11 (55%) cases, respectively. Five-year survival of the 16 available cases was 54%. Early clinical stage and medium-sized cell lymphoma were significantly (P < 0.05) better prognostic factors. Patients with GTCL exhibit distinct clinicopathological findings and prognoses from those with enteropathy-associated T-cell lymphomas. GTCL may be mainly derived from lamina propria and parafollicular T cells.

Clinicopathologic Analysis of Primary Gastrointestinal T-Cell Lymphoma with a Special Reference to Intestinal and Gastric Type in Japan.

Primary gastrointestinal T-cell lymphomas (PGITL) are very rare and heterogeneous diseases with poor prognosis. Their incidence and pathogenesis may vary with geographical locations. In this study, we aimed to define clinicopathologic and prognostic features of PGITL in Japan, non endemic area for celiac disease, with compared to those in Western countries. 79 patients with PGITL (41 gastric and 38 intestinal lymphoma cases) were enrolled. Epstein-Barr virus associated NK-/T-cell lymphoma and human T-cell leukemia virus type 1 associated adult T-cell leukemia/lymphoma cases were excluded from this study. Our intestinal cases (63%) were histopathologically characterized by monomorphic small-to medium-sized tumor cells, which appeared to be contrasted with higher incidence of pleomorphic appearance among Western patients, and by frequent expression of CD8, CD56, and cytotoxic molecules (CM) of those cells, which is consistent with immunohistochemical features of Western cases. Our gastric cases were unique in showing a wide range of histologic and immunophenotypical characteristics with a significantly higher inclusion of non-cytotoxic lymphoma than intestinal cases (33% vs. 9%, P=.0299). Approximately 62% of patients also showed pleomorphic appearance most frequently with CD4+CD8− and CD4–CD8-phenotype. In comparison with gastric patients, intestinal ones were significantly associated with several clinical factors to indicate poor prognosis such as poorer performance status (P=.0232), lower total protein level (P=.0018), and lower serum albumin level (P=.0303). Overall survival of intestinal cases were significantly inferior to that of gastric patients (P = 0.026), while no statistically significant differences were found in survival between CM-positive PGITL cases and –negative ones (P = 0.6). In univariate analysis, the factors that turned out to correlate significantly with survival were total protein level (<6.0g/dL) (HR, 3.696; 95% CI, 1.671 to 8.171; P=.0012), platelet level (<10.0 /μl) (HR, 10.706; 95% CI, 2.713 to 42.244; P=.0007), a higher than normal serum lactate dehydrogenase (LDH) level (HR, 2.574; 95% CI, 1.266 to5.231; P=.009), direct invasion or gastrointestinal perforation (HR, 2.079; 95% CI, 1.013 to 4.267; P=.046), bone marrow involvement of the disease (HR, 3.134; 95% CI, 1.136 to 8.651; P=.0274), performance status equal to or more than 2 (HR, 3.988; 95% CI, 1.763 to 8.882; P=.0007), the International Prognostic Index (IPI) more than low-intermediate (HR, 3.317; 95% CI, 1.304 to 8.441; P=.0119), Prognostic Index for T-cell lymphoma (PIT) scores more than 2 (HR, 8.166; 95% CI, 2.574 to25.904; P=.004), CD8 positivity (HR, 2.041; 95% CI, 1.032 to 4.037; P=.0404), and intestinal case (HR, 2.955; 95% CI, 1.493 to 5.849; P=.0019). Multivariate analysis with individual factors confirmed that PIT scores was a significant (HR, 6.622; 95% CI, 1.108 to 39.566; P=.0382) and platelet level (<10.0 /μl) was a marginally significant (HR, 5.343; 95% CI, .837 to 34.116; P=.0765) prognostic factors independent from other clinical factors. In conclusion, Japanese intestinal lymphoma patients were in keeping with characteristics of Enteropathy type T-cell lymphoma other than an association with celiac disease and monomorphic histology. However, gastric T-cell lymphomas were distinct from intestinal ones, and were separately considered.

Gastric T‐cell lymphoma with cytotoxic phenotype

Primary gastric lymphoma usually originates from B cells of mucosa-associated lymphoid tissue (MALT) infected with Helicobacter pylori. When T-cell lymphomas develop in the stomach, they usually occur in association with infection by human T-lymphotropic virus type 1 and gastric involvement of adult T-cell leukemia. Reported herein is a unique and informative case of gastric peripheral T-cell lymphoma with a cytotoxic phenotype that histologically mimicked, and had to be carefully distinguished from, MALT-type B-cell lymphoma. The patient, a 73-year-old woman, underwent a gastric endoscopy examination, and the histological findings suggested MALT-type gastric lymphoma. Analysis of the immunoglobulin heavy chain (IgH) gene and T cell receptor gamma (TCRgamma) gene revealed monoclonal rearrangement of the TCRgamma gene. The tumor cells exhibited mild atypia and immunoreactivity with anti-CD3, anti-CD8, anti-T-cell intracellular antigen-1, antigranzyme B and antiperforin antibodies, but not with anti-CD20, anti-CD10, and anti-CD79a antibodies. The case was finally diagnosed as gastric T-cell lymphoma with cytotoxic phenotype, and this was confirmed after surgical resection. In cases such as this, small biopsy specimens from the stomach should be examined carefully for low grade B-cell-type malignant lymphoma (MALT lymphoma), because sometimes the proliferating B cells can hide the truly malignant T cells, and rearrangement analysis is useful for diagnosing T-cell malignancy.

Long-term Remission of Primary Gastric T Cell Lymphoma Associated with Human T Lymphotropic Virus Type 1: A Report of Two Cases and Review of the Literature

Two cases of primary gastric T-cell lymphoma associated with human T lymphotropic virus type 1 (HTLV-1) are presented. Case 1 was a 54-year-old man who had multiple ulcerating tumors in the lower corpus and gastric antrum. Case 2, a 60-year-old man, showed a large ulcerating tumor in the upper corpus. Both patients were positive for serum anti-HTLV-1 antibody and for the monoclonal integration of HTLV-1 proviral DNA in the tumor cells by Southern blot analysis. The patients were thus diagnosed as having primary gastric T-cell lymphoma associated with HTLV-1 of stage II(1). Case 1 underwent total gastrectomy followed by chemotherapy, while Case 2 was treated by chemotherapy and radiotherapy. Both patients have been in complete remission for more than 4 years (96 months in Case 1 and 50 months in Case 2) after the treatments. Although primary gastric T-cell lymphomas associated with HTLV-1 is characterized by an extremely poor prognosis, the present cases suggest that in the early stage, long-term survival can possibly be achieved with appropriate treatments.

Primary Gastric T-cell Lymphoma without Human T-lymphotropic Virus type 1

Most primary gastric lymphomas are of B-cell origin and those of T-cell origin are very rare. We report a case of T-cell lymphoma without human T-lymphotropic virus type 1 (HTLV-1). A 73-year-old Japanese woman was admitted with epigastralgia and lumbago. Endoscopic examination visualized a submucosal tumor on the greater curvature of the cardia. Biopsy yielded a diagnosis of gastric malignant lymphoma. Lymph node swelling was recognized from the lesser curvature of the stomach to the lesion surrounding the aorta on computed tomography and ultrasonography, but no swelling of lymph nodes in the chest or superficial lymph nodes was seen. Total gastrectomy and local lymph node dissection was performed. HTLV-1 pro-viral DNA was negative in the resected specimen. Immunological findings in the resected specimen were positive in CD3, CD4, and negative in CD20, CD79α, and CD8. Gastric T-cell lymphoma without HTLV-1 was diagnosed. After the operation, chemotherapy was performed for lymph node swelling at the posterior of the pancreas head and surrounding the aorta. Two courses of chemotherapy (CHOP) yielded a complete response. The patient was well and disease free for 6 months, but she died 18 months after the operation with recurrence of lymph node swelling surrounding the aorta. Gastric T-cell lymphoma without HTLV-1 is associated with a poor prognosis in the literature.

Primary Gastric T-Cell Lymphoma: Clinicopathologic Features and Treatment Outcome.

Primary gastric lymphoma is the most frequent type of extranodal malignant lymphoma. The large majority of cases are of B-cell lineage, while primary gastric T-cell lymphomas are exceedingly rare and least well characterized malignancy and the clinical features have been described only in a few case reports. In this study, we conducted a retrospective analysis to investigate the natural history and the clinical outcome after treatment. Between 1990 and 2004, 444 patients with primary gastric lymphoma had been diagnosed at four institutes. Among these 444 patients, 17 patients (3.8%) were identified as primary gastric lymphoma with T-cell immunophenotype. The pathologic review was performed by two experienced hematopathologist to confirm T-cell lymphoma with typical immunohistochemical features including CD3. All of the patients were staged according to the Mushoff system. Among these 17 gastirc T-cell lymphoma patients, 14 patients were treated with combination chemotherapy including CHOP with or without radiotherapy. The two patients were treated with high-dose chemotherapy with autologous peripheral hematopoietic stem cell transplantation. The median age of the 13 male and 4 female patients was 49 years (range 22–76 years). The pathologic subtypes of these patients with T-cell immunophenotype were as follows; precursor T-lymphoblastic lymphoma in 2 patients, peripheral T-cell lymphoma, unspecified in 6 patients, NK/T cell lymphoma in 3 patients, and T-cell others in 5 patients. The many of the patients showed poor performance status (PS), and high LDH level (65%). 12 patients (71%) showed stage III or IV diseases, and 9 patients (53%) showed high or high-risk according to the International Prognostic Index (IPI). A response to treatment was observed in 8 patients (47%), with 6 Complete Response (CR) and 2 Partial Response (PR). The median Progression-Free Survival (PFS) and Overall Survival (OS) were only 5 months (95% C.I.; 4–6 months), and 14 months (95% C.I.; 0–189 months). 5 patients of these 17 patients (29%) presented only in stomach and regional lymph nodes. The patients with localized gastric T-cell lymphoma showed good therapeutic outcome (median PFS; 53 months, median OS; 123 months, p =0.024 by log-rank test). Other clinical factors including PS, age, and LDH did not show clinical significance in this series. Although our series had limitation to interpret due to small population, this study showed that the incidence of this subtype of T-cell gastric lymphoma was very rare, and poor prognosis, like as other T-cell lymphoma. However, about 30% of the patients had localized diseases limited to gastric area and regional lymph nodes with relative good prognosis. New therapeutic strategies need to improve the outcome of patients with gastric T-cell lymphoma.

Gastric T-cell lymphoma associated with hemophagocytic syndrome

BackgroundLymphoma-associated hemophagocytic syndrome (LAHS) occurs in mostly extra nodal non-Hodgkin's lymphoma. LAHS arising from gastrointestinal lymphoma has never been reported. Here we report a case of gastric T-cell lymphoma-associated hemophagocytic syndrome.Case presentationA 51-year-old woman presented with pain, redness of breasts, fever and hematemesis. Hematological examination revealed anemia. Gastroscopy revealed small bleeding ulcers in the stomach and the computed tomography scan showed liver tumor. She underwent total gastrectomy for gastrointestinal bleeding and the histopathology revealed gastric T-cell lymphoma. She continued to bleed from the anastomosis and died on the 8th postoperative day. Autopsy revealed it to be a LAHS.ConclusionsIf Hemophagocytic syndrome (HPS) occurs in lymphoma of the gastrointestinal tract, bleeding from the primary lesion might be uncontrollable. Early diagnosis and appropriate treatment are needed for long-term survival.

Coeliac disease and (extra)intestinal T-cell lymphomas: definition, diagnosis and treatment

Intestinal lymphomas encompass those lymphomas with a dominant or only localized occurrence in the intestinal tract. Coeliac disease is highly associated with enteropathy-associated T-cell lymphomas (EATLs). Coeliac disease-related lymphomas can appear at nodal or extranodal sites. EATL is often multifocal with ulcerative lesions, which explains the high perforation rate at presentation or during chemotherapy. Staging includes ear-nose-throat examination and CT scan of the chest and abdomen. Positron emission tomography (PET) scanning may be valuable. Accurate diagnosis based on endoscopic biopsies is preferable; if necessary, full thickness laparoscopic small-bowel biopsies are mandatory. Refractory coeliac disease (RCD) with aberrant T cells carries a high risk of development of EATLs. There is no satisfactory treatment for EATL, the only possibility of preventing EATL development in RCD being autologous bone marrow transplantation. EATLs can present in 20% of patients as extra-small-bowel T-cell lymphomas; such as subcutaneous panniculitis-like lymphoma, hepatosplenic γ/δ lymphoma, nodal as well as sinus, gastric or colon disease and extraintestinal T-cell lymphomas. The majority of EATLs present as large cell lymphoma CD[Formula: See Text] [Formula: See Text], CD[Formula: See Text] [Formula: See Text], CD[Formula: See Text] [Formula: See Text]; however, they also present as small cell lymphoma CD[Formula: See Text] [Formula: See Text], CD[Formula: See Text] [Formula: See Text], CD[Formula: See Text] [Formula: See Text]. Sometimes γ/δ lymphomas in CD are recognized. Work-up of EATL must include immunohistology, T-cell flow cytometry, T-cell rearrangement and adequate imaging with CT and PET scanning.

Non-HTLV-1-associated primary gastric T-cell lymphomas show cytotoxic activity: clinicopathological, immunohistochemical characteristics and TIA-1 expression in 31 cases.

Most primary gastrointestinal lymphomas are of B-cell origin and T-cell origin is very rare. Recent studies have suggested that human T-cell lymphotrophic virus type 1 (HTLV-1) may be involved in the development of primary gastric T-cell lymphoma. We analysed 31 patients with primary gastric T-cell lymphoma in south-west Japan, an area endemic for HTLV-1, and determined their phenotypes, genotypes, and HTLV-1 status. Here we present 31 cases of primary gastric T-cell lymphoma in a HTLV-1-endemic area in Japan and analyse the clinical status, histology, phenotype and virus status. The median age at onset of primary gastric T-cell lymphoma was 57 years with a gender ratio of M:F = 1.58:1. Six of the 31 primary gastric T-cell lymphoma cases had HTLV-1 proviral DNA (five males, one female), nine of the 31 cases were positive for anti-adult T cell leukaemia antibody, without examination of HTLV-1 proviral DNA (five males, four females), eight were non-HTLV-1-associated primary gastric T-cell lymphoma (four males, four females) and the other eight cases were unknown. Primary gastric T-cell lymphoma usually presented as a large ulcerated tumour at the corpus to the antrum and histologically consisted of anaplastic large cell type (n = 2), pleomorphic large cell type (n = 3), pleomorphic medium and large cell type (n = 14), pleomorphic medium cell type (n = 11), and angioimmunoblastic T-cell lymphoma type (n = 1). There were no clear macroscopic and microscopic differences between HTLV-1-associated and non-HTLV-1-associated primary gastric T-cell lymphoma. Most patients died within 2 years of diagnosis, and both types of primary gastric T-cell lymphoma (with and without HTLV-1) were associated with poor prognosis. Cytotoxic marker analysis showed that HTLV-1-associated lymphomas were negative for TIA-1, while non-HTLV-1-associated lymphomas were positive for TIA-1. Our results suggest that in HTLV-1-endemic areas, patients with HTLV-1-associated primary gastric T-cell lymphoma should be managed carefully and that TIA-1 seems to be useful for identifying the aetiology of this lesion.

Primary Gastric T-cell Lymphoma Associated with Human T-cell Leukemia Virus Type I shows 'Lymphoepithelial Lesions': Case report.

We describe an unusual case of adult T-cell leukemia/lymphoma (ATL) appearing in a 44-year-old female without leukemic change and systemic lymphadenopathy, which originated from the gastric wall and partially involved the paragastric lymph nodes. No other intraabdominal or intrathoracic organs, such as the liver, spleen, kidneys, adrenals, lungs, or bone marrow, were involved at the time of surgery. The clinical stage of this case was evaluated as IIE according to the Ann Arbor classification. Lymphoma cells were CD3+, CD4+, CD8-, and CD79a-. Southern blot analysis revealed monoclonal integration of proviral DNA from human T-cell leukemia virus type I. Interestingly, the lymphoma cells formed ‘lymphoepithelial lesions’ which are usually observed in gastric mucosa-associated lymphoid tissue (MALT) B-cell lymphomas. A rare pattern involving ATL cells and the presence of lymphoepithelial lesions in this case indicated a diagnostic pitfall of gastric MALT lymphoma.

Primary gastric apoptosis-rich T-cell lymphoma co-expressing CD4, CD8, and cytotoxic molecules

In contrast to primary gastric lymphomas of B-cell type, little is known about primary gastric T-cell lymphomas. We describe three cases with remarkably similar features: diffuse growth, epitheliotropism, medium too large cell size, high apoptotic rates, and a CD3+, CD4+, CD8+, CD45RO+ immunophenotype. Clonal TCRgamma gene rearrangement was shown in two cases. Epstein-Barr virus infection was excluded in two cases. Taking advantage of fresh-frozen material, we analyzed two cases further, revealing CD5-, CD16+, CD56-, CD57-, CD25+, CD30+, CD103 (alphaEbeta7)+, bcl-2 protein+, CD95+, CD95 ligand(L)-. CD95L, however, was detected in histiocytic and fibroblastoid by stander cells. The lymphomas expressed granzyme B, perforin, and the TIA-1 antigen in various combinations. All three cases had a very unfavorable clinical course characterized by local recurrence and/or dissemination to other epithelial sites, leading to death within 6-12 months after the initial diagnosis despite surgery and aggressive antineoplastic treatment. These data suggest a novel variant of peripheral T-cell lymphoma operationally characterized as primary gastric, apoptosis-rich, CD103+, EBV-, T-cell lymphoma co-expressing CD4, CD8, CD16 and cytotoxic molecules.

Genotype Configuration in a Case of Primary Gastric Lymphoma with T-cell Phenotype

T-cell malignant lymphoma of the gastrointestinal tract is rare. The genotype of gastric T-cell lymphoma remains unclear. The aim of this study was to elucidate the pathogenesis of a case of primary gastric T-cell lymphoma by using cytogenetics and molecular biology. Gastric biopsy specimens and lymphoma cells in the ascites were examined by immunocytology, cytogenetic analysis, and Southern blot analysis. The histological diagnosis of the gastric lymphoma was diffuse large cell type. T-cell markers were positive in immunocytochemistry of the gastric lymphoma cells and in FACS analysis of lymphoma cells in the ascites. All lymphoma cells in the ascites had complex abnormal karyotypes containing t(8;14)(q24;q32). Southern blot analysis revealed rearrangement of the IgH and C-MYC genes of the lymphoma cells in both the stomach and the ascites, but no comigration of the C-MYC with the JH locus could be detected. The TCR-β and -γ genes were in their germ-line configurations. In this patient, although the phenotype was T-cell lymphoma, the karyotype t(8;14)(q24;q32) and genotype had the characteristics of B-cell lymphoma. The unique B-cell genotype configuration and the C-MYC activation suggested that the cellular origin of this rare case of malignant lymphoma with a T-cell phenotype was quite immature lymphocytes.