Primary Epicardial Malt Lymphoma : A New Physiopathologic Entity Mimicking a Pericardial Compressive Syndrome
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ABSTRACT:
SUBMITTER: Grandmougin D
PROVIDER: S-EPMC8572141 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Primary Sjogren's syndrome (pSS) confers increased risk for non-Hodgkin lymphoma (NHL) development. Two common polymorphisms, the c. 677C?>?T and c. 1298A?>?C, of the methylene-tetrahydrofolate reductase (MTHFR) gene, an enzyme essential in DNA synthesis and methylation, have been associated with susceptibility to NHL. Herein, we tested the hypothesis that MTHFR variants contribute to pSS-related lymphomagenesis. 356 pSS patients, of whom 75 had MALT and 19 non-MALT NHL and 600 healthy controls were genotyped for the detection of MTHFR polymorphisms. DNA methylation levels were assessed by pyrosequencing of the LINE-1 retroelement promoter in DNA from 55 salivary gland tissues from pSS patients. DNA double-strand breaks were determined in peripheral blood mononuclear cells from 13 pSS patients, using comet assay. ?nalysis according to lymphoma subtype revealed increased frequency of c. 677C?>?T TT genotype and T allele, as well as reduced prevalence of the c. 1298A?>?C C allele in the pSS non-MALT group compared to controls and patients without NHL. MTHFR c. 677C?>?T TT genotype was associated with reduced DNA methylation levels, while MTHFR c. 1298A?>?C AC genotype with reduced DNA double-strand breaks levels. MTHFR variants may be involved in SS non-MALT NHL development, through contribution to defective DNA methylation and genomic instability.
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Project description:The stomach is the most frequent site of extranodal lymphoma. Gastric lymphoma originating from mucosa-associated lymphoid tissue (MALT) is typically a low-grade, B-cell neoplasia strongly associated with Helicobacter pylori (H. pylori) infection. Only certain H. pylori strains in some predisposed patients determine lymphoma development in the stomach, according to a strain-host-organ specific process. The clinical presentation is poorly specific, symptoms ranging from vague dyspepsia to alarm symptoms. Similarly, different endoscopy patterns have been described for gastric lymphoma. H. pylori eradication is advised as first-line therapy in early stage disease, and complete lymphoma remission is achieved in 75% of cases. Neoplasia stage, depth of infiltration in the gastric wall, presence of the API2-MALT1 translocation, localization in the stomach, and patient ethnicity have been identified as predictors of remission. Recent data suggests that H. pylori eradication therapy may be successful for gastric lymphoma treatment also in a small subgroup (15%) of H. pylori-negative patients. The overall 5-year survival and disease-free survival rates are as high as 90% and 75%, respectively. Management of patients who failed to achieve lymphoma remission following H. pylori eradication include radiotherapy, chemotherapy and, in selected cases, surgery.
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Project description:Primary hepatic lymphoma (PHL) presenting with obstructive jaundice is rare and can mimic a preoperative diagnosis of cholangiocarcinoma. We should consider PHL in patients with radiological hepatic disease with normal serum alpha-fetoprotein and carcinoembryonic antigen levels, and elevated lactate dehydrogenase. We present the case of a 67-year-old male with no significant medical history presented with abdominal pain, jaundice, fever, and abnormal liver function tests. Abdominal sonography and computed tomography scan suggested a diagnosis of obstructive jaundice and cholangitis due to cholangiocarcinoma (Klatskin tumor). A subsequent liver biopsy diagnosed PHL, and the patient was treated with combination chemotherapy, including rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP). PHL should be considered in patients presenting with biliary obstruction.
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Project description:BACKGROUND:This case represents the first report of malignant primary cardiac tumour in a patient with Lynch Syndrome associated with MSH2 pathogenic variant. CASE PRESENTATION:A 57-year-old woman with previous ovarian cystadenocarcinoma was admitted to the emergency room for hematic pericardial effusion. Multimodal diagnostic imaging revealed two solid pericardial vascularized masses. After pericardiectomy, the final histological diagnosis was poorly differentiated pleomorphic sarcomatoid carcinoma. During follow-up she developed an ampulla of Vater adenocarcinoma. Genetic analysis identified an MSH2 pathogenic variant. CONCLUSION:This case contributes to expand the tumour spectrum of Lynch syndrome, suggesting that MSH2 pathogenic variants cause a more complex multi-tumour cancer syndrome than the classic Lynch Syndrome. In MSH2 variant carriers, symptoms such as dyspnoea and chest discomfort might alert for rare tumours and a focused cardiac evaluation should be considered.
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Project description:BackgroundPrimary lymphomas of the gastrointestinal tract are rare, accounting for only 1 to 4% of malignancies arising in the stomach, small intestine, or colon. The stomach is the most common extranodal site of lymphoma and gastric mucosa-associated lymphoid tissue (MALT) lymphoma accounts for 40% of primary gastric lymphoma. Gastric MALT lymphoma reaches its peak incidence between 50 to 60?years of age, therefore, it is rarely encountered in pediatric population. The presenting symptoms of gastric MALT lymphoma are usually nonspecific and primary perforation of gastric MALT lymphoma is uncommon.Case presentationA 12?year-old female presented with iron deficient anemia developed gastric perforation. Emergency laparoscopic repair of the perforation was performed and tissue pathology showed gastric MALT lymphoma infiltration. Helicobacter pylori eradication and radiotherapy were sequentially performed. Complete remission was achieved at two months after radiotherapy. To our best knowledge, she is the youngest patient with gastric MALT lymphoma reported in the literature.ConclusionIron deficient anemia is a common presenting manifestation of malignancies in adulthood. In pediatric population, iron deficient anemia is usually caused by nutritional deficient or blood loss. In this case report, we present a teenaged female without previous gastric ulcer history who presented with a rare gastric tumor and an uncommon primary perforation. Even if there is an uncertainty about the exact diagnosis prior to the surgery, the strategy of stomach-preserving therapy by laparoscopy for primary perforation was successful and provided a good quality of life.
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Project description:Primary gastric lymphoma (PGL) represents a rare pathology, which can be easily misdiagnosed because of unspecific symptoms of the digestive tract. Histologically, PGL can vary from indolent marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) to aggressive diffuse large B-cell lymphoma (DLBCL). During the years, clinical trials revealed the important role of Helicobacter pylori (H. pylori) in the pathogenesis of gastric MALT lymphoma. Infection with Helicobacter pylori is an influential promoter of gastric lymphomagenesis initiation. Long-term studies revealed that eradication therapy could regress gastric lymphomas.
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