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1/27. Multiple lymphomatous polyposis of the gastrointestinal tract is a heterogenous group that includes mantle cell lymphoma and follicular lymphoma: analysis of somatic mutation of immunoglobulin heavy chain gene variable region.

    Multiple lymphomatous polyposis (MLP) is characterized by multiple polyps involving long segments of the gastrointestinal (GI) tract. MLP is thought to represent mantle cell lymphoma (MCL) of the GI tract; however, some cases of follicular lymphoma (FL) of the GI tract are found with a multiple polypoid appearance. In the present study, to clarify the cellular origin of MLP, clonal immunoglobulin heavy chain (IgH) gene rearrangement of four cases with MLP was amplified by polymerase chain reaction (PCR) and analyzed for the presence of somatic mutation. The IgH variable (VH) region sequences of three cases (CD5 CD10- cyclin d1 ) showed a little somatic mutation compared with the closest published germline. The other case (CD10 CD5- cyclin d1-) was highly mutated and showed intraclonal heterogeneity (ongoing somatic hypermutation). These data indicate that three of the cases with MLP are derived from pregerminal center B cells (mantle zone B cells) and one case with MLP from germinal center B cells. Our study suggests that MLP is a heterogenous group that includes MCL and FL.
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keywords = lymphomatous
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2/27. gastrointestinal stromal tumors treated by laparoscopic surgery: report of three cases.

    Three cases of gastrointestinal stromal tumors (GIST) were treated by a laparoscopic wedge resection of the stomach. The tumor characteristics were confirmed to be nonepithelial, nonlymphomatous, nonmyogenic, and nonneurogenic gastrointestinal neoplasms with an uncertain origin which were CD34-positive and actin- and S-100-negative. The malignant potential was estimated based on the mitotic figures and growth rates. The results suggest that laparoscopic surgery is an adequate strategy for gastric submucosal tumors including GIST, and also indicates this technique to be a curative, safe, and minimally invasive procedure for both diagnosis and treatment.
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keywords = lymphomatous
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3/27. Increased incidence of follicular lymphoma in the duodenum.

    The incidence of indolent lymphomas in the lymph nodes and extranodal regions is quite different. Follicular lymphoma (FL) is most common in the nodes, and it seems to be least common in the gastrointestinal (GI) tract, where mucosa-associated lymphoid tissue lymphoma arises most frequently. The authors report that the incidence of FL is unexpectedly high in the duodenum compared with other portions of the GI tract. FL was detected in only eight of 222 cases of GI lymphoma (3.6%). However, five cases of FL arose in the duodenum, which accounted for 38.5% of 13 duodenal lymphomas. Only in two patients did FL arise in either the stomach or the colorectum, and in the remaining patients FL was widespread with lymphomatous polyposis. Duodenal FL was composed of neoplastic follicles with small cleaved cells in dominance, and the immunophenotype of the lymphoma cells was CD10 , BCL-2 , CD20 , CD75 , CD79 , CD3-, CD5-, cyclin d1-, CD23-, and CD45RO-. All the patients were women age 37 to 66 years (average age, 52.4 yrs). In all patients the lymphoma was present around the ampulla of vater, and four of five patients showed multiple small-size polyps. Although lymphoma cell infiltration was confined to the submucosa in the four patients examined, the regional lymph nodes were involved partially in two patients without distant metastasis. All patients are alive at 2 to 50 months of follow up (average, 27 mos), which is comparable with the prognosis for indolent nodal lymphomas. These results suggest that the duodenum has a distinct background of histogenesis of the lymphomas and that biopsy specimens from the duodenum with multiple polyps should be examined carefully.
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keywords = lymphomatous
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4/27. Endoscopic ultrasonography in multiple lymphomatous polyposis.

    Two cases of multiple lymphomatous polyposis (MLP) are presented, involving different segments of the gastrointestinal tract. Both cases display the characteristic clinical and pathologic features of MLP. In addition, we were able to document, for the first time, the endoscopic ultrasonographic findings in this disease. This new ancillary diagnostic technique was found to be very helpful in the evaluation of the structural changes of the wall of the gastrointestinal tract and in the detection of affected lymph nodes.
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keywords = lymphomatous
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5/27. A rare case of multiple lymphomatous polyposis with widespread involvement of the gastrointestinal tract.

    Multiple lymphomatous polyposis (MLP) is an uncommon type of primary non-Hodgkin gastrointestinal (GI) B-cell lymphoma characterized by the presence of multiple polyps along the GI tract. Malignant cells of MLP have mantle cell characteristics and thus are considered to be the counterpart of the mantle cell lymphoma (MCL) in the GI tract. Since 1961, no more than 70 well-documented cases have been published. We report the case of 53-year-old man diagnosed as having MLP. The patient presented with diffuse abdominal pain, chronic lower GI bleeding, peripheral lymphadenopathy, and weight loss. The lymphomatous polyps extended from the esophagus to the rectum, with bone marrow infiltration. Immunohistologic findings were characteristic of MCL. The patient was treated with a combined cyclophosphamide, vincristine, and prednisone chemotherapy regimen, resulting in a partial response.
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ranking = 1.2
keywords = lymphomatous
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6/27. Peripheral T-cell lymphoma of AILD (angioimmunoblastic lymphadenopathy with dysproteinemia) type involving gastrointestinal tract. A morphologic, phenotypic and genotypic study.

    A case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) which showed widespread involvement of the gastrointestinal tract is reported. A lymph node biopsy specimen showed the characteristic histological features of AILD. During the progression of the illness, lymphomatous lesions developed in the gastrointestinal tract, complicated by cytomegalovirus infection. A double immunoenzymatic study using a combination of Ki-67 antibody and antibodies against surface antigens demonstrated that CD3 , CD4 , and/or T-cell receptor (TCR) beta cells were predominant (67-68%) among the population of proliferating Ki-67% cells, rather than CD8 or CD22 cells. Clonal rearrangement of the TCR beta chain gene was also detected. These findings provide further evidence for the neoplastic nature of lesions of this type, and the diagnosis of peripheral T-cell lymphoma.
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keywords = lymphomatous
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7/27. Multiple lymphomatous polyposis of the gastrointestinal tract--a report of two cases with immunohistochemical studies.

    We describe two cases of multiple lymphomatous polyposis in the gastrointestinal tract from the esophagus to the rectum. Clinical findings, histopathologic and immunohistochemical findings in paraffin embedded tissue are discussed. It is important to recognize this rare form of gastrointestinal lymphoma because of the prognostic and therapeutic implications.
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keywords = lymphomatous
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8/27. Multiple lymphomatous polyposis of the gastrointestinal tract.

    CONTEXT: Gastrointestinal multiple lymphomatous polyposis is a rare type of malignant lymphoma that has aggressive biological behavior, early systemic dissemination and poor prognosis. It is considered to be a manifestation of non-Hodgkin lymphoma and represents the gastrointestinal counterpart of mantle cell nodal lymphoma. OBJECTIVE: A case of gastrointestinal multiple lymphomatous polyposis is presented and the anatomopathological, clinical, diagnostic and treatment aspects of this unusual neoplasia are discussed. CASE REPORT: The patient was a 59-year-old white male with a complaint of asthenia, night sweating, alteration in intestinal habit and weight loss over the preceding two months. The physical examination showed pallid mucosa and a palpable mass in the epigastrium and mesogastrium. endoscopy of the upper digestive tract showed the presence of gastric and duodenal polyps. An opaque enema showed multiple polypoid lesions, especially in the cecum. A rectal biopsy revealed infiltration of the mucosa and submucosa by diffuse lymphoma consisting of small cleaved cells. Immunohistochemical study showed lymphocytes that expressed the antibody CD20 (L-26) and light-chain kappa (k) immunoglobulin, but not light-chain lambda (l) immunoglobulin. The patient presented a condition of acute intestinal obstruction with the presence of a mesenteric mass formed by agglutinated lymph nodes that surrounded the proximal ileum, thereby obstructing its lumen. He was submitted to a segmental enterectomy and gastrotomy with excisional biopsies of the gastric polypoid lesions. After two cycles of chemotherapy there was a worsening of the general state, with an increase in the dimensions of the abdominal masses and sepsis, accompanied by progressive respiratory insufficiency, leading to death.
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keywords = lymphomatous
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9/27. Non-multiple lymphomatous polyposis form of mantle cell lymphoma in the gastrointestinal tract.

    Mantle cell lymphoma (MCL) comprises 2.5%-7% of all non-Hodgkin's lymphomas, and the gastrointestinal tract is involved in about 20% of cases. Multiple lymphomatous polyposis (MLP) is an uncommon disease that is regarded as the intestinal form of MCL. We present a rare case of gastrointestinal MCL without MLP, and demonstrate that rituximab was effective for the treatment of this patient. A 61-year-old man presented with continuous diarrhea and hematochezia for a period of 5 months. Superficial lymph nodes were not palpable, but both tonsilla were enlarged. The level of soluble interleukin (IL)2-receptor was 3480 U/ml (normal <500 U/ml). colonoscopy showed diffuse redness with erosion, without observation of any venous capillary, with these findings continuing from the rectum to the ileum. Upper gastrointestinal endoscopy showed a slightly rough gastric mucosal surface, and chicken-skin like mucosa was observed in the second portion of the duodenum. Small-to-medium size lymphoma cells were seen histologically from the tonsilla to the rectum. The lymphoma cells were immunohistochemically positive for CD5, CD20, CD79a, and cyclin d1. polymerase chain reaction analysis revealed a chromosomal translocation t(11;14)(q13;q32) in the bcl-1 gene. We diagnosed this as a case of MCL from these findings. For treatment, the patient received a total of ten courses of combination chemotherapy consisting of cyclophosphamide (1000 mg), doxorubicin (70 mg), vincristine (2 mg) and prednisolone (50 mg) (CHOP), which led to a partial remission. However, 2.5 years later, massive infiltrations of the lymphoma cells were found in the colon and stomach. As the infiltrating lymphoma cells expressed CD20 molecules on their surfaces, the patient was treated with a chimeric anti-CD20 monoclohal antibody, rituximab, which showed significant efficacy, and a second partial remission was achieved.
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keywords = lymphomatous
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10/27. Gastrointestinal involvement and multiple lymphomatous polyposis in mantle-zone lymphoma.

    The clinical, histologic, and immunologic features of three cases of lymphoma presenting with gastrointestinal symptoms and involving the gastrointestinal tract were studied. Two of the cases had involvement of long segments of the bowel with polypoid lesions, a rare presentation of gastrointestinal lymphoma referred to as multiple lymphomatous polyposis (MLP). All cases were classified as mantle-zone lymphoma (MZL), a follicular variant of intermediate lymphocytic lymphoma (ILL) characterized by the proliferation of small atypical lymphoid cells as wide mantles surrounding benign-appearing germinal centers. The patients were typical of other patients with MZL in that they were male, middle age or older, and their clinical courses were not aggressive despite the presence of disease at an advanced stage. Our review of the literature suggests that there is an inordinate number of cases of MZL with gastrointestinal involvement. We also note that most reports of cases of MLP have described histologic lesions remarkably similar to what we have observed. We conclude that MZL may have predilection for involvement of the gastrointestinal tract, that this involvement is often in the manner of MLP, and that most cases of MLP probably have MZL or ILL.
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keywords = lymphomatous
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