GI Mantle Cell Lymphoma

Mantle cell lymphoma (MCL) is a rare and aggressive non-Hodgkin's B cell lymphoma characterized by the translocation t(11;14) (q13;32) and overexpression of CCND1 . MCL is immunophenotypically identified as CD20+ , CD5+ , CyclinD1+, CD43+ , CD10- , BCL6- , and CD23- . It is often distinguished from B cell lymphomas of germinal center cell origin by the absence of CD10 expression. Here we report the unique clinicopathologic features of a patient with CD10+ MCL with gastrointestinal involvement and review current literature identifying this unique immunophenotype...

Mantle cell lymphoma is a rare and aggressive non-Hodgkin lymphoma with a high propensity for extranodal involvement, typically seen in the liver, marrow, and gastrointestinal system. Being an FDG-avid disease and a sensitive modality for detecting both nodal and extranodal disease, FDG PET/CT is recommended in the workup of mantle cell lymphoma. We present a rare pattern of cutaneous involvement of the head-neck region in a patient with mantle cell lymphoma, which can be described on FDG PET/CT as a "hood sign...

INTRODUCTION: Although it usually involves extranodal sites such as the gastrointestinal tract in more than 80% of cases, mantle cell lymphoma is considered a rare cause of gastrointestinal bleeding, especially severe and life-threatening bleeding. PATIENT CONCERN: A 60-year-old man with peptic ulcer disease, who presented with severe upper gastrointestinal (GI) bleeding and large gastric ulcer. DIAGNOSIS: Primary gastric mantle cell lymphoma...

BACKGROUND AND AIM: This study aimed to investigate the relationship between the histological type of colorectal lymphoma and its endoscopic features. METHODS: We retrospectively analyzed patients with primary colorectal lymphoma who were diagnosed using colonoscopy and biopsy specimens at the National Cancer Center Hospital, Tokyo, Japan. The lesions were macroscopically classified into the following types via colonoscopy: polypoid, ulcerative, multiple lymphomatous polyposis, diffuse, and mixed...

Bruton's tyrosine kinase inhibitors have changed the treatment landscape for chronic lymphocytic leukemia (CLL), mantle cell lymphoma (MCL) and lymphoplasmacytic lymphoma dramatically. In 2019, acalabrutinib was approved by the US FDA for the treatment of treatment-naive and relapsed/refractory CLL and MCL. Acalabrutinib monotherapy was found to be effective and safe in CLL patients. In ASCEND and ELEVATE treatment-naive studies, acalabrutinib monotherapy and the combination with obinutuzumab demonstrated improved efficacy and an acceptable safety profile...

Mantle cell lymphoma is typically an aggressive, rare form of non-Hodgkin lymphoma that arises from cells originating in the "mantle zone." Here, we describe a case of mantle cell lymphoma associated with gastrointestinal bleeding, which was worked up as a possible gastrointestinal lesion or intraluminal pathology. The patient presented with symptomatic microcytic anemia associated with a positive guaiac fecal test. However, after an extensive workup to elucidate the symptomatic anemia's etiology, mantle cell lymphoma associated with a positive guaiac test was the culprit...

We report the first pedigree of Lynch syndrome bearing a germ-line MSH2 missense mutation c.1808A>T (Asp603Val). Until now, this missense mutation, in exon 12 of MSH2, was identified as a variant of unknown significance in the International Society for Gastrointestinal Hereditary Tumours database. In vitro induction mutagenesis experiments indicated that the MSH2 mutant protein (Asp603Val) is easily degraded in embryonic stem cells, albeit there is no clinical information concerning this mutant. Our pedigree includes four patients with Lynch syndrome-associated malignancies and clinically matches the Amsterdam II criteria...

Primary pancreatic lymphomas (PPLs) are rare, and the histological classification of these tumors is difficult. To accurately diagnose and determine the appropriate treatment for PPLs, sufficient sample amounts are necessary. Here, we report a 73-year-old man with a primary pancreatic mantle cell lymphoma. Histological samples were obtained via endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA). The tumor cells predominantly composed of atypical small to medium round cells, with diffuse immunoreactivity of CD20 and cyclin D1...

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Mantle cell lymphoma (MCL), a type of B-cell non-Hodgkin's lymphoma, is a rare and aggressive disease with a poor prognosis due to its advanced presentation at diagnosis. It is characterized by a translocation in the Bcl-1 gene, which results in overexpression of cyclin D1. MCL is frequently seen in the form of multiple lymphomatous polyposis (MLP) in which innumerable polyps are observed in the gastrointestinal (GI) tract. In rare instances, MCL presents a single mass. The most common presentation involves male patients in their sixties, with generalized lymphadenopathy, extranodal involvement, and B symptoms (night sweats, fever, and weight loss)...

BACKGROUND/AIM: Malignant lymphoma (ML) cases with overlapping gastrointestinal (GI) lesions are often encountered. We aimed to elucidate the importance of examining the GI tract in patients with ML and assess the overlap rate. PATIENTS AND METHODS: We analysed 190 patients diagnosed with GI MLs. We compared the overlap rates among the different histopathological types. RESULTS: Twenty-five (13.2%) patients had overlapping GI lesions in more than two segments...

We present a 63-year-old man with a medical history of hepatocellular carcinoma who underwent orthotopic liver transplant 10 years prior on long-term immunosuppressive therapy. The patient presented to the clinic with diarrhea, and the workup revealed mantle cell lymphoma. Mantle cell lymphoma is an extremely rare finding in transplanted livers. It is essential to include mantle cell lymphoma, along with a broad differential, during the workup of diarrhea in post-transplant patients.

The pathogenesis of autoimmune gastritis (AIG) remains unclear. In addition, it is difficult to follow the process of AIG onset endoscopically. Leukemic non-nodal mantle cell lymphoma (MCL) was newly added as a subtype of MCL in the fourth revised edition of the World Health Organization (WHO) classification (2017). Here, we report a case of AIG associated with the progression of leukemic non-nodal MCL. A 74-year-old woman who had been followed up in a nearby hospital for chronic B-cell lymphoproliferative disorder with no treatment for six years presented with fever and fatigue in the previous one month...

Primary classical Hodgkin lymphoma (CHL) in the colon is exceedingly rare and shares many histologic features with other lymphoproliferative disorders in the gastrointestinal tract. Here we report a case of CHL forming a sigmoid mass. An elderly man with a past medical history of mantle cell lymphoma presented with constipation. Imaging revealed an ulcerated, circumferential mass in the sigmoid colon. Endoscopic biopsy of the mass showed ulcerated colonic mucosa with an underlying diffuse mixed inflammatory infiltrate admixed with Hodgkin and Reed-Sternberg cells...

BACKGROUND: Zanubrutinib is a Bruton's tyrosine kinase inhibitor that has been recently licensed in refractory mantle cell lymphoma and under assessment in phase 3 clinical trials for other B cell malignancies. To date, there are no reported cases of hepatotoxicity secondary to zanubrutinib. We report the first case of severe liver injury due to zanubrutinib. CASE PRESENTATION: A 56-year-old Caucasian male with a history of relapsed lymphoplasmacytic lymphoma was admitted to the hospital with new-onset jaundice, choluria, and pruritus for 10 days...

PURPOSE OF THE REVIEW: Primary GI lymphomas of B cell origin are a diverse group of lymphomas. In this article, we provide an overview of the diagnosis, pathologic and molecular features, and management of these varied lymphomas. RECENT FINDINGS: The most common primary GI lymphomas are diffuse large B cell lymphoma (DLBCL) and marginal zone lymphomas (MZL), but follicular lymphomas (FL), mantle cell lymphomas (MCL), post-transplant lymphoproliferative disorders (PTLD), and Burkitt lymphoma of the GI tract also occur...

Mantle cell lymphoma is a rare and aggressive type of B-cell non-Hodking lymphoma. It can compromise the gastrointestinal tract, sometimes developing an entity known as multiple lymphomatous polyposis. It develops more frequently in males in the sixth decade of life, presenting heterogeneous clinical patterns. The diagnostic is endoscopic with histological confirmation. Currently, the treatment is chemotherapy, reserving surgical exploration of the abdominal cavity for complicated cases with acute abdomen. We present the case of a 51-year-old woman who underwent emergency surgery to treat peritonitis due perforation of a multiple lymphomatoid polyposis, an unreported atypical complication...

The gastrointestinal (GI) tract is home to a significant portion of the immune system, which interacts daily with the antigenic milieu of its contents. Therefore, the presence of white blood cells within the walls of the GI tract upon histologic examination is a familiar sight on GI biopsies-both in health and disease. The GI tract is the most common site of extranodal lymphomas, most of which are B-cell neoplasms. Here, we review common and uncommon B-cell neoplasms of the GI tract - extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), mantle cell lymphoma, duodenal-type follicular lymphoma, diffuse large B-cell lymphoma, plasmablastic lymphoma, EBV-positive mucocutaneous ulcer, and post-transplant lymphoproliferative disorders - with special focus on literature published during the past five years...

BACKGROUND: Mantle cell lymphomas are aggressive, mature B-cell neoplasms characteristically showing overexpression of cyclin D1. Although lymphadenopathy is the most common presentation, involvement of extranodal sites including bone marrow, peripheral blood, liver, gastrointestinal system, and Waldeyer ring is also seen frequently. Soft tissue localization is extremely rare. It has blastoid and pleomorphic subtypes associated with aggressive course. CASE DESCRIPTION: We describe a 74-year-old man who had been diagnosed 3 years previously with "mantle cell lymphoma-blastoid type" and presented 3 months ago with a giant mass in the right lower extremity that enlarged rapidly up to 15 cm in a few months...

Immunoglobulin light chain (AL) amyloidosis is characterized by the deposition of amyloid fibers derived from pathologic immunoglobulin light chains. Although systemic plasma cell neoplasms are the most common cause of AL amyloidosis, a subset of cases is caused by B-cell lymphoproliferative disorders such as lymphoplasmacytic lymphoma or extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue. Recently, SOX11-negative IGH hypermutated mantle cell lymphoma (MCL) is recognized to show frequent plasmacytic differentiation and indolent clinical course...