Mucosa-associated lymphoid tissue lymphoma of the esophagus coexistent with bronchus-associated lymphoid tissue lymphoma of the lung

Non-Hodgkin's lymphoma very rarely involves the esophagus, occurring in less than 1% of patients with gastrointestinal lymphoma. A few cases of mucosa-associated lymphoid tissue (MALT) lymphoma of the esophagus have been reported in the English literature. To our knowledge, there has been no report of MALT lymphoma of the esophagus coexistent with bronchus-associated lymphoid tissue lymphoma (BALT) of the lung. This report details the radiological and clinical findings of this first concurrent case.     

Mucosa-associated lymphoid tissue lymphoma in the conjunctiva of a child

Marginal zone lymphoma of the mucosa-associated lymphoid tissue (MALT) occurring in the conjunctiva has yet not been described in pediatric patients. We present a case of a 10-year-old girl with a MALT lymphoma involving the conjunctiva. The tumor consisted of plasma cells and marginal zone cells with discrete epitheliotropism. Immunohistochemical studies revealed positivity for CD20 and cytoplasmic immunoglobulin light chain restriction. Polymerase chain reaction-based molecular analysis of the infiltrate showed a monoclonal rearrangement for the hypervariable complimentary determining region III immunoglobulin region; whereas, a polyclonal pattern was seen for the T-cell receptor chain. Extensive further examination, including molecular techniques, revealed that the lymphoma was restricted to the conjunctiva (stage IA) and was not associated with any specific infection. The patient was treated with surgery and additional local cryotherapy. After 15 months of follow-up, the patient remains in complete remission.     

Low grade gastric B-cell lymphoma of mucosa associated lymphoid tissue in immunocompromised patients

An increased incidence of non-Hodgkin's lymphoma is seen in patients with immunodeficiency from any cause. The majority of these are high grade B-cell lymphoma and most are associated with the Epstein-Barr virus (EBV). In post-transplant lymphoma/lymphoproliferative disorders the tumour may regress following reduction of immunosuppression but in AIDS the lymphomas show a characteristic aggressive course and poor prognosis. We describe low grade B-cell gastric lymphoma of mucosa associated lymphoid tissue (MALT) in three immunocompromised patients (two post-transplant, one HIV positive). In each case, the tumour showed classical morphological features of gastric MALT lymphoma and was not associated with EBV. Helicobacter pylori was identified in each case. Clinical follow-up suggests that the behaviour in these tumours is similar to that seen in MALT lymphomas in immunocompetent patients and not typical of the lymphomas usually associated with immunosuppression. Although the finding of MALT lymphoma in immunosuppressed patients might be coincidental, the association of some MALT lymphomas with autoimmune disease suggests that dysregulation of the immune system might play a role in the pathogenesis of these tumours.     

Mucosa-associated lymphoid tissue lymphoma: molecular pathogenesis and clinicopathological significance

Mucosa-associated lymphoid tissue (MALT) lymphoma is a low-grade tumor closely associated with chronic inflammation such as that of Helicobacter pylori gastritis, Sjogren's syndrome, and Hashimoto's thyroiditis. Tumor regression by H. pylori eradication alone is well known in gastric MALT lymphoma, but some tumors occur in the absence of pre-existing chronic inflammation. The understanding of MALT lymphoma biology has significantly improved, and recurrent cytogenetic alterations have been detected. These include the trisomies 3 and 18, and the translocations t(11;18)(q21;q21), t(1;14)(p22;q32), t(14;18)(q32;q21), and t(3;14)(p14.1;q32). At least some of these alterations result in the constitutive activation of the nuclear factor (NF)-kappaB pathway, and may exert anti-apoptotic action. Apoptosis inhibitor 2-MALT lymphoma-associated translocation 1 (API12-MALT1) fusion, resulting from t(11;18)(q21;q21), is specific to, and is the most common in, MALT lymphomas, and its clinicopathological significance has been studied extensively. The focus of the present review is on the recent progress made in elucidating MALT lymphomagenesis and its clinicopathological impact, especially in terms of the effect of API2-MALT1 fusion on this unique tumor.     

乳腺粘膜相关型淋巴瘤临床病理观察

目的：探讨乳腺粘膜相关性淋巴瘤（MALT-ML）的病理特征。方法：对4例乳腺MALT-ML的手术根治及（或）活检标本做常规石蜡切片、HE染色和免疫组化ABC法标记。结果：4例乳腺MALT-ML中2例为CCL细胞型,1例为CCL细胞型向母细胞样转化,另1例炎单核样B细胞型。4例均显示B细胞单克隆性及滤泡克隆化和淋巴上皮病变。结论：乳腺MALT-ML有与其他部位MALT-ML相似的形态特征。     

Low-grade B cell lymphoma of mucosa-associated lymphoid tissue in the thymus of a patient with rheumatoid arthritis

The majority of thymlc lymphomas are either lymphoblastic lymphoma, large B cell lymphoma or Hodgkin's disease, and other types of non-Hodgkin lymphoma are rare. A case of low-grade B cell lymphoma of mucosa-associated lymphoid tissue (MALT) In the thymus is reported. A 55-year-old Japanese female with a history of rheumatoid arthritis (RA) complained of back pain. A mediastinal tumor was identified by computerized tomography and magnetic resonance imaging, and the thymus was resected through median sternotomy. The solid and nodular tumor had several small satellite extensions and was completely confined to within the thymus. Hlstologically, monotonous medium-sized centrocyte-like cells occupied the medulla of the thymus and Infiltrated Hassan's corpuscles (lymphoepithellal lesions). Immunohistochemically, tumor cells were positive for CD20 and CD79a. IgA and kappa light chain restriction were also found in plasmacytoid cells in the tumor. Clonal rearrangement of the immunoglobulin heavy chain gene was demonstrated by polymerase chain reaction. This case was diagnosed as MALT-type low-grade B cell lymphoma In the thymus. This is the first report of low-grade B cell lymphoma In the thymus associated with RA. As autoimmune diseases are known to be associated with lymphoid neoplasms, It is suggested that the RA played an Important role in the development of malignant lymphoma in this case.     

老年人胃粘膜相关淋巴组织型淋巴瘤

研究胃粘膜相关淋巴组织型淋巴瘤的临床病理,免疫表型及与幽门螺杆菌的关系,方法：应用免疫组织地进行免疫分型,对ＭＡＬＴ型淋巴瘤和淋巴组织反应性增生进行鉴别,用Ｇｉｅｍｓａ染色显示幽门螺杆菌,对患者进行随该。结论：胃的粘膜相关淋巴组织型淋巴瘤与幽门螺杆菌的感染有关,临床在化疗时应用抗菌素。     

Comparison of cutaneous sarcoidosis with systemic sarcoidosis: a retrospective analysis

Objective: To investigate clinical characteristics and treatment effect of sarcoidosis with cutaneous lesions in Chinese patients, and to compare them with previous works. Methods: Retrospective analysis was conducted based on clinical manifestations, systemic examinations and treatment of biopsy-proved 36 patients with sarcoidosis with cutaneous lesions in our hospital since 2000. Patients were divided into cutaneous sarcoidosis (CS) group without systemic involvement and systemic sarcoidosis (SS) group with systemic involvement according to whether extracutaneous systems were involved. Results: Male to female ratio was 1:4.1 in total 36 patients. Average age of onset was (43.6±15.8) years old in CS group and (54.4±11.5) years old in SS group. The most common cutaneous lesions were papulonodules (41.7%) and frequently found in limbs (61.1%). There were 26 patients in SS group, and lung was the most common organ with systemic involvement, followed by lymph nodes. In SS group, elevation of inflammatory parameters and evident changes of chest radiologic examination were often observed. 72.2% patients were treated with glucocorticoid and the overall therapeutic efficacy rate was 48.4%. The therapeutic efficacy in CS group (80%) was significantly higher than SS group (33.3%). Papulonodules type had better response to therapy and usually resolved after treatment. Lupus pernio type was resistant to treatment. Conclusion: Sarcoidosis occurs more frequently in females. Lung is the most commonly affected extracutaneous organ in SS patients. CS patients have better response to therapy than SS patients. Types of cutaneous lesions and existence of systemic involvement are related to prognosis of cutaneous lesions.     

Early lymphoma coexisting with reactive lymphoid hyperplasia of the stomach

A 55-year-old woman with a small focus of malignant lymphoma, 6 mm in diameter, arising within an area of reactive lymphoid hyperplasia (RLH) of the stomach is reported. The diagnosis of lymphoma was suspected by the preoperative endoscopic biopsy. This is the case of this association with an unusually small lymphoma in the early stage, in support of the view that there may be an intimate histogenetic relationship between lymphoma and RLH.     

Plasmablastic lymphoma of the stomach in an HIV‐negative patient

Plasmablastic lymphoma (PBL) is a rare B‐cell neoplasm with an aggressive clinical behavior that predominantly occurs in the oral cavity of human immunodeficiency virus (HIV)‐positive patients. However, it has recently been recognized that PBLs can also affect individuals without HIV infection, and suggested that these neoplasms show different clinicopathological characteristics between HIV‐positive and ‐negative patients. Herein we describe a case of gastric PBL in a female HIV‐negative patient. The tumor was composed of a diffuse and cohesive proliferation of large neoplastic cells, which resembled immunoblasts or plasmablasts with a starry sky appearance. Immunophenotypically, the neoplastic cells were diffusely positive for CD138, MUM1, IgM, and BOB‐1, and negative for CK, LCA, CD3, CD20, CD79a, Pax5, kappa, lambda, CD30, ALK, S‐100, HMB‐45, MPO, and HHV‐8. The MIB‐1 index was nearly 100%. Epstein‐Barr virus‐encoded RNA in situ hybridization was negative. A monoclonal immunoglobulin heavy chain gene rearrangement was detected in polymerase chain reaction (PCR) and heteroduplex analyses. A combination of PCR‐based analysis of immunoglobulin gene rearrangement and immunohistochemistry can be useful to substantiate the diagnosis by utilizing routine paraffin‐embedded tissue sections, because PBL in the setting of extra‐oral localization and immunocompetence is a diagnostic challenge, given its rarity, morphology, and absence of CD20 expression.     

MUC1 mucin expression in follicular dendritic cells and lymphoepithelial lesions of gastric mucosa-associated lymphoid tissue lymphoma

Membrane-associated mucin MUC1 is expressed in various adenocarcinoma cells and active T lymphocytes. We tried to find out whether MUC1 is expressed in gastric mucosa-associated lymphoid tissue (MALT) lymphoma lesion. MUC1 was not expressed in infiltrating T lymphocytes; however, MUC1 was found on the cell surface of follicular dendritic cells (FDC) of germinal centers and in the epithelial cytoplasm of lymphoepithelial lesion (LEL) of the lymphoma, which were immunohistochemically detected by monoclonal antibodies DF3 and MY.1E12. MUC1 was also expressed in the FDC of control cases (gastrectomy specimen containing reactive lymphoid follicles, n = 10, MUC1/ DF3, 100%; MUC1/MY.1E12, 40%), and FDC in MALT lymphomas (n = 59) showed lower MUC1 expression rates (MUC1/ DF3, 32%; MUC1/MY.1E12, 0%) than the control (P < 0.001). Lymphoepithelial lesion in the low-grade MALT lymphomas (n = 23) showed a higher MUC1/DF3 expression rate (30%) than those in the high-grade MALT lymphomas (n = 36; 6%; P < 0.05). T lymphocytes in the surface mucosa were more frequent in MALT lymphoma (91.4 +/- 80.6/unit area) than those in the control (20.0 +/- 23.6) (P < 0.001). S100-positive dendritic cells around LEL were more frequent in the low-grade (19.0 +/- 9.4/unit area) than in the high-grade (11.7 +/- 9.7) (P < 0.005). This study demonstrated MUC1 mucin expression on FDC for the first time. Mucosa-associated lymphoid tissue lymphoma, especially low-grade, shows immunologically active state, where FDC MUC1 expression may be suppressed by some factors released from lymphoma cells. Further study to elucidate the pathogenetic role of MUC1 in MALT lymphoma is necessary.     

Mucosa-associated lymphoid tissue of the thymus hyperplasia vs lymphoma.

In the thymus, the relationship between lymphofollicular hyperplasia and mucosa-associated lymphoid tissue (MALT)-type lymphoma is uncertain. We analyzed 14 cases with a diagnosis of thymic follicular hyperplasia in patients with connective tissue disease (n = 2), myasthenia gravis (n = 11), or both (n = 1). In 11 cases, well-defined reactive lymphoid follicles were surrounded by a continuous layer of medullary epithelial cells. A polyclonal rearrangement of the immunoglobulin heavy chain gene (IgH) was observed. In 3 cases, ill-defined lymphoid follicles with sheets of centrocytic-like B cells disrupting the medullary cytokeratin epithelial network were observed on certain sections. These cells expressed the phenotypic features of memory B cells with CD20, CD79a, and bcl-2 positivity and CD5, CD10, CD23, and bcl-6 negativity, and a monoclonal rearrangement of the IgH gene was detected. Appropriate sampling, cytokeratin staining, and molecular analyses may help to identify early MALT-type lymphoma developing in the setting of thymic lymphofollicular hyperplasia.     

Monocytoid B-cell lymphoma: morphological variants and relationship to low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue

Twenty-eight cases of monocytoid B-cell lymphoma of lymph nodes and 16 lymph node metastases of primary gastric lymphomas, mostly low-grade B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) type were investigated morphologically and immunohistochemically. Both groups showed the same morphological and immunohistochemical features: diagnostically important sites of infiltration were the sinuses and the marginal zones. The tumour cells were either medium-sized or small. The cytoplasm stained grey with Giemsa and was sometimes rather pale. In imprints the grey colour of the cytoplasm was a characteristic feature. The medium-sized cell type was more frequent; in one third of the cases it was combined with a prominent lymphoplasmacytic component from the same clone, and it resembled the monocytoid B-cells of the sinuses. The small cell type was less common, was not combined with a lymphoplasmacytic component and more closely resembled marginal zone cells. The difference was underlined by the negative reaction with the monoclonal antibody Ki-B3 in the small cell type, which, conversely, was positive in the medium-sized cell type and in the monocytoid B-cell reaction of the sinuses. Both of these cell types, however, showed a granular reaction with the new monoclonal antibody Ki-Mlp. The morphological and immunohistochemical parallels are arguments in favour of the assumption that monocytoid B-cell lymphoma is the nodal equivalent of low-grade B-cell lymphoma of MALT type. This is further supported by the fact that in nine of our 28 cases of monocytoid B-cell lymphoma, lymphomas were found simultaneously or subsequently in organs of the MALT. Monocytoid B-cell lymphoma must be differentiated from an infiltration that occurs in the form of clusters of monocytoid B-cells in other low-grade B-cell lymphomas, especially in immunocytoma with a high content of epithelioid cells.     

Autopsy case of lymphoplasmacytic lymphoma with a large submucosal tumor in the stomach

An autopsy case of lymphoplasmacytic lymphoma with a large submucosal tumor in the stomach is presented. The patient was a 77-year-old woman with gastric lymphoma associated with Waldenstrom's macroglobulinemia of IgM-lambda type. Diagnosis was initially mucosa-associated lymphoid tissue (MALT) lymphoma of the stomach, because gastric biopsy specimens showed epitheliotropic proliferation (lymphoepithelial lesion) of the lymphoma cells. Postmortem examination revealed a large gastric lymphoma with metastatic foci in the esophagus, larynx, trachea, lungs, spleen and lymph nodes. The bone marrow was also involved. Lymphoma cells consisted of small lymphocytoid cells occasionally admixed with blast-like large cells and a large number of plasmacytoid or plasma cells. Centrocyte-like cells were not found. Lymphoepithelial lesions were not conspicuous in autopsy specimens. Immunohistochemically, lymphoma cells reacted with CD20, CD45, CD79a, anti-IgM, anti-lambda protein and anti-BCL-2, but not with CD5, CD10, CD23 or CD38. Based on these findings, the revised diagnosis of the present case was lymphoplasmacytic lymphoma, and it highlighted the differential diagnostic problem from marginal zone B-cell lymphoma of MALT type.     

Malignant lymphoma of mucosa-associated lymphoid tissue

Lymphomas of the gastrointestinal tract, salivary glands, lung and thyroid are grouped together as tumours arising in mucosa-associated lymphoid tissue. The great majority of them are of B-cell origin but distinctive T-cell lymphomas are also recognized in the gastrointestinal tract. These lymphomas tend to remain localized for prolonged periods but, whereas the B-cell group respond favourably to local therapy, the T-cell group are associated with severe morbidity and their overall prognosis is extremely poor. Accepted histological classifications of non-Hodgkin's lymphomas are difficult to apply to these tumours. In this paper their morphological features are reviewed; recent findings based on immunohistochemistry and DNA analysis are presented; and the biological behaviour of these tumours is discussed insofar as they offer insight into mucosal immunological mechanisms.