B-cell Lymphoma Of Mucosa-associated Lymphoid Tissue Research Articles

Primary hepatic extranodal marginal zone B-cell mucosa-associated lymphoid tissue lymphoma treated by laparoscopic partial hepatectomy: a case report

BackgroundPrimary hepatic extranodal marginal zone B-cell mucosa-associated lymphoid tissue (MALT) lymphoma is very rare, so it is difficult to diagnose preoperatively. And there is no established treatment for hepatic MALT lymphoma. We report herein a case of primary hepatic MALT lymphoma treated by laparoscopic partial hepatectomy, and discuss the usefulness of laparoscopic hepatectomy for a rare liver tumor.Case presentationThis patient was a woman in her 60s, who was diagnosed preoperatively as having synchronous liver metastasis from sigmoid colon cancer; therefore, laparoscopic partial hepatectomy was performed. She had a good course after the operation and was discharged on postoperative day 12. However, she was diagnosed pathologically as having primary hepatic MALT lymphoma. A bone marrow biopsy was also performed, and then she was finally diagnosed as having limited-stage primary hepatic MALT lymphoma. She received no postoperative treatment and showed no recurrence for 4 years postoperatively.ConclusionsWe experienced the good result of the patient with limited-stage primary MALT lymphoma treated by laparoscopic partial hepatectomy. Liver tumors are sometimes misdiagnosed by imaging examinations alone. Laparoscopic hepatectomy has been widespread recently as a minimally invasive procedure, and it may be useful for both diagnosis and treatment.

Anti-CD40L therapy prevents the formation of precursor lesions to gastric B-cell MALT lymphoma in a mouse model.

Mucosa-associated lymphoid tissue (MALT) lymphoma is a B-cell tumour that develops over many decades in the stomachs of individuals with chronic Helicobacter pylori infection. We developed a new mouse model of human gastric MALT lymphoma in which mice with a myeloid-specific deletion of the innate immune molecule, Nlrc5, develop precursor B-cell lesions to MALT lymphoma at only 3months post-Helicobacter infection versus 9-24 months in existing models. The gastric B-cell lesions in the Nlrc5 knockout mice had the histopathological features of the human disease, notably lymphoepithelial-like lesions, centrocyte-like cells, and were infiltrated by dendritic cells (DCs), macrophages, and T-cells (CD4+ , CD8+ andFoxp3+ ). Mouse and human gastric tissues contained immune cells expressing immune checkpoint receptor programmed death 1 (PD-1) and its ligand PD-L1, indicating an immunosuppressive tissue microenvironment. We next determined whether CD40L, overexpressed in a range of B-cell malignancies, may be a potential drug target for the treatment of gastric MALT lymphoma. Importantly, we showed that the administration of anti-CD40L antibody either coincident with or after establishment of Helicobacter infection prevented gastric B-cell lesions in mice, when compared with the control antibody treatment. Mice administered the CD40L antibody also had significantly reduced numbers of gastric DCs, CD8+ and Foxp3+ T-cells, as well as decreased gastric expression of B-cell lymphoma genes. These findings validate the potential of CD40L as a therapeutic target in the treatment of human gastric B-cell MALT lymphoma. © 2023 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.

MALT LYMPHOMA IN PAROTID GLAND ASSOCIATED WITH SJÖGREN'S SYNDROME

Mucosa-associated lymphoid tissue (MALT) lymphoma is a subtype of non-Hodgkin lymphoma, often affecting the parotid glands in Sjögren syndrome (SS). MALT lymphoma is an indolent disease frequently located in mucosal or nonmucosal extranodal sites. A 63-year-old woman complained of a nodular growth near her ear. Upon palpation, a nodular mass was observed in proximity to the anterior lobe of the left parotid gland. She reported dry mouth feeling lasting 2 years, hoarseness, dryness of the eyes and vaginal mucosa, tiredness, and body pain. Parotid ultrasonography and magnetic resonance imaging, minor salivary gland biopsy, and ophthalmologic evaluation were obtained. After 2 inconclusive ultrasound-guided needle biopsies, the lesion was removed by partial parotidectomy. Immunohistochemical analysis showed B-cell MALT lymphoma in the parotid gland, associated with SS. The patient was referred to a hematology oncologic center for evaluation and follow-up was established every 6 months.

Association of Pulmonary MALT Lymphoma and Sarcoidosis

Introduction Sarcoidosis is a systemic granulomatous disease of unknown etiology characterized by the chronic inflammation. Mucosa associated lymphoid tissue (MALT) lymphoma is a low-grade marginal zone B-cell lymphoma of MALT often etiologically associated with chronic inflammation in the underlying organ. A rare association between sarcoidosis and MALT lymphoma has been reported. Case Description We present the case of a 75-year-old woman who presented with chronic cough and dyspnea of several years' duration. She was found to have fluctuating lung infiltrates on serial imaging studies. A bronchial biopsy revealed chronic inflammation, and no evidence of infection. She was then treated with oral steroids with a transient benefit. However, the infiltrate and symptoms recurred, therefore 8 months later, she underwent repeat imaging. This showed a relapse of the infiltrate and enlarged mediastinal lymphadenopathy. The lung infiltrate was biopsied (percutaneously). This demonstrated a MALT lymphoma. Imaging studies done for staging showed a large retroperitoneal lymph node. A biopsy of this node revealed non-caseating granulomas, consistent with sarcoidosis. Chemotherapy with bendamustine and rituximab was initiated for therapy of the MALT lymphoma. Discussion A rare association between pulmonary MALT lymphoma and underlying inflammation caused by sarcoidosis has been previously described. In our patient, sarcoidosis was diagnosed in a biopsy of a retroperitoneal lymph node, but we suspect that there is likely involvement of the mediastinal nodes. Disclosures Rao: Bayer & Daiichi Sankyo:Consultancy.Daniel:GSK:Other: Paid speaker for COPD medications.

A Case Report of Non-Hodgkin Low-grade B-cell Mucosa-associated Lymphoid Tissue Lymphoma Presenting as a Suspected Endodontic Lesion

Extranodal mucosa-associated lymphoid tissue lymphoma is a rare oral disease. This case report describes a 71-year-old woman who presented for endodontic evaluation and treatment for swelling on the right posterior palatal. The case details the evaluation and diagnostic processes and treatment course. The case report draws attention to the importance of performing a biopsy, having proper sampling, and the additional steps sometimes required to arrive at a definitive diagnosis. This case report is the first to document a definitive low-grade B-cell mucosa-associated lymphoid tissue lymphoma mimicking endodontic pathology. The case shows the importance of proper examination, clinical imaging, and pathologic testing to arrive at a definitive diagnosis. It highlights that collaboration with other medical and dental specialties is essential for appropriate diagnosis and treatment and describes the “buttonhole” diagnostic strategy.

S1798 Composite Lymphomas in the Colon: Two Separate Entities, Treated as One

INTRODUCTION: Composite lymphoma (CL) is defined as two histologically distinct variants of malignant lymphoma occurring in the same organ or mass. Our case describes an asymptomatic patient with two extranodal B-Cell lymphomas discovered after routine colonoscopy. Further, we discuss pathogenesis and treatment philosophy of follicular lymphoma and marginal cell lymphoma. CASE DESCRIPTION/METHODS: A 62 year old Japanese female with multiple medical morbidities including cirrhosis and hypothyroidism presented for surveillance colonoscopy. On presentation, patient denied any fever, chills, night sweats, unintentional weight loss, hematochezia, or melena. Colonoscopy revealed multiple sessile polyps, 2−5 mm in size. H&E and immunohistochemical staining of specimens demonstrated grade 3A follicular lymphoma with 100% follicular pattern and positron emission tomography/computed tomography (PET/CT) demonstrating a prominent, isolated pericaval lymph node. Per NCCN guidelines for follicular lymphoma grade 3A, stage II (non-contiguous), observation with follow up PET/CT and colonoscopy was recommended. Repeat colonoscopy after 6 months revealed multiple polyps with demonstrating extranodal marginal zone b-cell lymphoma of Mucosa-Associated Lymphoid Tissue (MALT). The diagnosis of MALT type lymphoma was rendered based on histologic patterns of infiltration, the cellularity of the infiltrate, their locations, presence of clear cells, and immunostaining. Of note, repeat PET/CT was negative for lymphadenopathy. 6 months later, another colonoscopy was performed, and 6 more polyps were biopsied. Histological examination was consistent with tubular adenomas and follicular lymphoid proliferation. Given that she was clinically stable with minimal disease, plan was to continue monitoring with colonoscopy and PET/CT annually. DISCUSSION: CLs are defined as 2 or more morphologically and/or immunophenotypically different components in the same anatomic site. They can arise synchronously or metachronously. CLs are rare, with a frequency ranging from 1 to 4.7% of lymphoma cases. More rarely, CL has seldom been witnessed in the gastrointestinal tract. To our knowledge, this is the first case reporting composite follicular lymphoma and extranodal marginal zone b-cell lymphoma of MALT in the colon. Current literature suggests the components of CLs progress as separate entities and treatment should be focused on the component with the least favorable prognosis.

Primary pulmonary extranodal marginal zone lymphoma/low grade B-cell lymphoma of MALT type combined with well-differentiated adenocarcinoma.

We describe a rare case of extranodal marginal zone/low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) combined with areas of well-differentiated adenocarcinoma. In addition, the MALT lymphoma was synchronously systemic, with involvement of the lung, stomach and duodenum.

Mucosa-Associated Lymphoid-Tissue Lymphoma of the Cecum and Rectum: A Case Report.

A colonic mucosa-associated lymphoid-tissue (MALT) lymphoma is relatively rare compared to lymphomas of the stomach or small intestine. We present a case of a MALT lymphoma in the cecum and rectum found during screening colonoscopy. A 54-year-old female, who had undergone right-breast-conserving surgery with axillary dissection due to an invasive ductal carcinoma and a left-breast excisional biopsy due to microcalcification following adjuvant chemoradiation therapy 3 years earlier, was found to have 3-mm-sized smooth elevated lesions in both the cecum and rectum. No pathologic lesion or lymphadenopathy was found at any other site, but chronic gastritis negative for Helicobacter pylori infection was found. The polyps were removed by using an endoscopic biopsy and revealed an extra nodal marginal zone B-cell MALT lymphoma, showing positive for CD3 and CD20 by immunohistochemical staining. The patient underwent close observation without any additional treatment and has shown no evidence of recurrence as of her last visit.

Endoscopic Findings of Gastric Extranodal Marginal Zone B-Cell Mucosa-Associated Lymphoid Tissue Lymphoma.

Endoscopic Findings of Gastric Extranodal Marginal Zone B-Cell Mucosa-Associated Lymphoid Tissue Lymphoma.

Bone marrow flow cytometry in staging of patients with B-cell non-Hodgkin lymphoma.

BackgroundBone marrow biopsies are routinely performed for staging patients with B-cell non-Hodgkin lymphoma (NHL). In addition to histomorphological studies, ancillary tools may be needed for accurate diagnosis. We investigated the clinical utility of multiparameter flow cytometric examination of bone marrow aspirates.MethodsA total of 248 bone marrow specimens from 232 patients diagnosed with B-cell NHL were examined. Monoclonal antibodies directed against CD19, CD20, CD10 (or CD5), and κ and λ immunoglobulins were used. Multi-stage sequential gating was performed to select specific cells of interest, and the results were compared with bone marrow histology.ResultsThe concordance rate between histomorphology and flow cytometry was 91.5% (n=227). Eight cases (3.2%) were detected by flow cytometry alone and were missed by histomorphology analysis, and 6 of these 8 cases showed minimal bone marrow involvement (0.09-2.2%). The diagnosis in these cases included large cell lymphoma (n=3), mantle cell lymphoma (n=3), and mucosa-associated lymphoid tissue (MALT) lymphoma (n=2). Thirteen cases were histopathologically positive and immunophenotypically negative, and the diagnoses in these cases included diffuse large cell lymphoma (n=7), T-cell/histiocyte-rich large B-cell lymphoma (n=2), anaplastic lymphoma kinase (ALK)-positive large B-cell lymphoma (n=1), follicular lymphoma (n=1), MALT lymphoma (n=1), and unclassifiable lymphoma (n=1).ConclusionsMulti-color flow cytometry can be a useful method for assessing bone marrow in staging NHL and also plays a complementary role, especially in detecting small numbers of lymphoma cells.

An Unusual Case of Marginal Zone B-Cell Lymphoma

Introduction: To present a case of a marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) presenting as a small bowel stricture. Methods: Case report and literature review. Results: A 49-year-old Ghanaian man with prior atrial septal defect repair and intermittent constipation presented with abdominal pain, nausea, vomiting, and lack of bowel movements, but passing flatus. He denied fevers, night sweats, weight loss, prior abdominal surgery, and any medication use including ACE inhibitors or non-steroidal anti-inflammatory drugs. Labs were unremarkable, CT of the abdomen and pelvis showed a partial small bowel obstruction (SBO) without a clear transition point, and an upper GI series was normal. The patient’s symptoms resolved with conservative management; however, 2 days later he was re-admitted with recurrent symptoms. CT showed recurrent partial SBO with a transition point in the left lower quadrant. Capsule endoscopy subsequently showed few scattered sessile polypoid lesions just proximal to a stricture in the small bowel; the capsule did not pass through this area. Double-balloon enteroscopy was pursued and showed a high-grade discrete stricture with erythematous and fibrotic mucosa in the proximal ileum with adjacent nodular, atrophic, and hypervascular mucosa. Pathology revealed Helicobacter pylori (H. pylori) gastritis and marginal zone B-cell lymphoma of MALT. Discussion: Small intestine MALT lymphoma is an uncommon entity seen in about 12.6-18.8% of all primary small intestine lymphomas. There are only a few case reports and case series in the literature describing small intestine MALT lymphoma, which were diagnosed after patients presented with abdominal pain, obscure gastrointestinal bleeding, abdominal distension, gastric outlet obstruction, SBO, intussusception, and perforation. On endoscopy, small intestine MALT lymphoma most commonly presents as ulcerative (66.7%) or polypoid (26.7%) lesions, with only 1 case reported as presenting with a stricture. Compared to other types of small intestine lymphomas, MALT lymphoma overwhelmingly presents at earlier stages and have very favorable prognoses. While gastric MALT lymphoma associated with H. pylori, usually responds with eradication, remission of small intestine MALT lymphoma after H. pylori eradication is less clearly demonstrated, and chemotherapy is often necessary. Conclusions: Although rare, small intestine MALT lymphoma should remain in the differential diagnosis for SBO and small bowel strictures.

A case of mucosa-associated lymphoid tissue lymphoma of the sigmoid colon presenting as a semipedunculated polyp.

Mucosa-associated lymphoid tissue (MALT) lymphomas are characterized by lymphoepithelial lesions pathologically. Colonic MALT lymphomas are relatively rarer than lymphomas of the stomach or small intestine. Endoscopically, colonic MALT lymphoma frequently appears as a nonpedunculated protruding polypoid mass and/or an ulceration in the cecum and/or rectum. We report a unique case of a colonic MALT lymphoma presenting as a semipedunculated polyp. A 54-year-old man was found to have a 2-cm semipedunculated polyp in the sigmoid colon during screening colonoscopy. The polyp was removed by endoscopic mucosal resection. Histologic examination of the resected polyp revealed diffuse epithelial infiltration by discrete aggregates of lymphoma cells. We diagnosed the tumor as low-grade B-cell MALT lymphoma by immunohistochemical staining.

Eradication Therapy in Helicobacter pylori-negative, Gastric Low-grade Mucosa–associated Lymphoid Tissue Lymphoma Patients

To assess the remission rate of gastric low-grade B-cell mucosa-associated lymphoid tissue lymphoma after an eradication therapy in Helicobacter pylori-negative patients. We performed a systematic review with pooled analysis of published studies. Data were analyzed according to: (1) number of H. pylori-negative patients treated with only eradication therapy; (2) number of patients in whom the complete lymphoma remission was achieved; and (3) the method used to exclude H. pylori infection. Overall, 11 studies with 110 patients met the inclusion criteria for this pooled analysis. H. pylori infection was excluded in all studies with at least 3 different diagnostic tests. Eradication therapy achieved a complete lymphoma regression in 17 (15.5%; 95% confidence interval, 8.7-22.2) patients. Eradication therapy is successful in a small but distinct subgroup of H. pylori-negative patients with low-grade mucosa-associated lymphoid tissue lymphoma. On the basis of the generally indolent behavior of this neoplasia, before resorting to aggressive, costly, and potentially more toxic oncologic therapies, it would seem reasonable to attempt eradication therapy in all patients, irrespective of their H. pylori status.

MALT lymphoma in labial salivary gland biopsy from Sjögren syndrome: importance of follow-up in early detection

Mucosa-associated lymphoid tissue (MALT) lymphomas are known to occur in Sjögren syndrome (SS) patients, but reported cases in labial salivary glands (LSG) are rare. We report a case of 60-year-old female patient with SS who developed MALT lymphoma in the labial salivary glands during a 2-year time interval when she was participating in the Sjögren's International Clinical Collaborative Alliance, an ongoing longitudinal multisite observational study funded by the National Institutes of Health of the United States. At follow-up exam, LSG biopsy showed atypical diffuse infiltration by mononuclear cells of variable size and atypical nuclei affecting the whole specimen with destruction of glandular architecture, leading to a diagnosis of B-cell MALT lymphoma. Computerized tomography and bone marrow biopsy failed to show additional evidence of disease. Clinical, serologic, ocular, histologic and immunohistochemical findings are presented. A "watch and wait" policy was adopted with regular examinations.

Primary B-Cell Mucosa-Associated Lymphoid Tissue Lymphoma Presenting as Hematochezia: A Multidisciplinary Approach to Treatment

Purpose: A 64-year-old Hispanic female presented with a three day history of dark and fresh blood mixed with her stools and diffuse intermittent abdominal pain. She denied any family history of GI related cancers or the use of non-steroidal anti-inflammatory drugs. She had never had a colonoscopy. Her physical exam revealed a vertical scar from a cesarean section. Her hemoglobin was 11.9 gm/dL and her white blood cell count was 7.7 k/μL. Colonoscopy revealed blood throughout the entire colon and a large 4 cm soft ulcerated submucosal mass in the cecum with a patent ileocecal valve. CT scan confirmed a large mass in the cecum with a diameter of 4.7 × 4.2 cm. The patient had a laparoscopic right hemicolectomy. The pathology specimen revealed engorged vessels and a constricted luminal center filled with mucoid tan material. Immunostaining and microscopic appearance revealed marginal zone B-cell mucosa associated lymphoid tissue (MALT) lymphoma involving the colon, appendix and lymph nodes in the fatty tissue with negative resection margins. MALT lymphoma is an extranodal lymphoma arising in various epithelial tissues including the stomach, salivary glands, lung, small bowel, and thyroid. Primary malignant lymphomas of the colon are rare neoplasms and account for approximately 0.2% of colorectal malignancies. MALT lymphoma involving the cecum accounts for only 9.2% of lymphoma lesions in the colon and the gross appearance of ulcerations occurs in 11.9% of cases. With a multidisciplinary approach to the treatment course, including resection of the primary tumor, patients have a good overall prognosis.Figure: No Caption available.Figure: No Caption available.

MALT Lymphoma of Caecum Presenting as Acute Intestinal Obstruction: A Case Report

Mucosa-associated lymphoid tissue lymphomas (MALT lymphoma) are well known, but colonic MALT lymphomas are extremely rare. We report the first case of mucosa-associated lymphoid tissue (MALT) lymphoma in the cecum from India presenting as acute intestinal obstruction with mass in right iliac fossa showing diffuse thickening of ascending colon and caecum with obliterated lumen along with nodular mass on CECT scan of abdomen. On video colonoscopy, circumferential ulcerated growth was seen at cecum, and the rest of the colon was normal. Multiple biopsies from growth suggested malignancy. Right hemicolectomy was performed. The histopathology of resected specimen demonstrated low-grade B-cell MALT lymphoma of the cecum, and the depth of invasion was up to the muscular layer. This was confirmed by immunohistochemistry.

A parotid gland lymphoma presenting with steroid-responsive recurrent sialadenitis: a case report

Parotid gland located lymphomas are rarely seen. The most common lymphomas involving salivary gland include B-cell MALT (mucosa-associated lymphoid tissue) lymphomas. Of them, extranodal marginal zone B-cell lymphomas are the most frequently seen pathologies. They usually present with painless swelling of the parotid gland. The diagnosis is often based on parotidectomy. In this article, we report a 46-year-old male patient who was admitted with recurrent sialadenitis attacks which responded well to steroid treatment for three years, but recur after the cessation of the therapy. With the initial diagnosis of recurrent parotitis, the patient was scheduled for total parotidectomy for the definitive diagnosis and treatment. When the frozen-section of specimens during superficial parotidectomy revealed lymphoma, the operation was terminated.

Konjonktivanın MALT Lenfoması

Mucosa associated lymphoid tissue (MALT) lymphoma (marginal zone B-cell lymphoma of MALT according to the World Health Organization classification) is one of the low- grade non-Hodgkin's lymphomas. The majority of nonHodgkin's lymphomas of the ocular adnexa are extranodal marginal zone B-cell lymphomas of the mucosa associated lymphoid tissue, also known as MALT lymphoma. In 8% of cases, it arises from the conjunctiva. Lymphoid tumors of the conjunctiva may appear as isolated neoplasms or a part of a systemic disease. In this study, we presented a case of primary conjunctival lymphoma and discussed the etiology, diagnosis and treatment with reference to previous studies and reviews.

Cryoglobulinaemia related to Sjogren's syndrome or HCV infection: differences based on the pattern of bone marrow involvement, lymphoma evolution and laboratory tests after parotidectomy

The relationship of cryoglobulinaemia with lymphoproliferation of mucosa-associated lymphoid tissue (MALT) as risk factors for lymphoma evolution in SS remains to be clarified. The different biologic background of SS-related cryoglobulinaemia as compared with cryoglobulinaemia linked to HCV infection was clarified by different clinical and biologic approaches. B-cell clonal expansion was analysed in the bone marrow of 27 consecutive cases with primary SS and mixed cryoglobulinaemia, HCV unrelated, in comparison with 55 HCV-related patients with cryoglobulinaemic vasculitis (CV) without SS. The results were related to the possible occurrence and localization of B-cell lymphoma in the single case. Secondly, the prevalence of mixed cryoglobulinaemia was investigated in 41 unselected patients with primary SS showing either parotid myoepithelial sialadenitis (MESA) or a frank B-cell non-Hodgkin's lymphoma. Thirdly, the levels of serum cryoglobulins and RF were followed in one patient with primary SS, CV and parotid B-cell lymphoma of MALT after bilateral subtotal parotidectomy. A polyclonal pattern of B expansion in the bone marrow was significantly more frequent in SS-related (19/27 cases) than in HCV-related cryoglobulinaemia (19/55) (P = 0.003). Cryoglobulins were positive in a fraction of patients with SS and malignant lymphoma or with parotid MESA (13/18 and 7/23, respectively), whereas MALT involvement by the lymphoproliferative disorder was the rule. Finally, the levels of serum cryoglobulins and RF markedly decreased in the SS patient undergoing bilateral subtotal parotidectomy. Lymphoproliferation of MALT appears as the biologic background of cryoglobulinaemia in SS, differently from HCV-related cryoglobulinaemia.

Marginal Zone B-cell Lymphoma of MALT in Small Intestine Associated with Amyloidosis: A Rare Association

A 62-yr-old man presented with a 5-yr history of intermittent abdominal distention and pain. These symptoms persisted for several months and subsided without treatment. A diagnosis of suspected small bowel lymphoma was made based on plain radiograph and computerized tomogram findings, and he was referred to our institution for further evaluation. Segmental resection of the small intestine was performed and the diagnosis of marginal zone B-cell lymphoma associated with amyloidosis was made. This is the first case of marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) in the small intestine associated with amyloidosis in Korea.