Primary Low-grade B-cell Lymphoma Research Articles

Analysis of the response time to involved-field radiotherapy in primary gastrointestinal low-grade B-cell lymphoma

BackgroundEarly-stage primary gastrointestinal (GI) low-grade B-cell lymphoma shows good therapeutic response to primary radiotherapy. However, there is no clear guideline for the evaluation of response to radiation therapy currently. The aim of this study was to analyze the relationship between the best response time and the clinical course after radiotherapy.MethodsPatients who underwent radiotherapy for treatment of primary GI low-grade B-cell lymphoma from September 2007 to December 2018 at Seoul St. Mary’s Hospital were included. Early responders were defined by best response within 6 months after radiotherapy, and delayed responders after 6 months. Clinical and pathological factors associated with delayed response and survival analyses were performed to investigate the recurrence and survival during follow-up.ResultsA total of 43 patients were evaluated and the number of gastric mucosa-associated lymphoid tissue and duodenal follicular lymphoma was 36 and 7, respectively. All of 43 patients showed complete remission to radiotherapy and the best response time after radiotherapy was a median of 3 months. There were 8 delayed responders with a median duration of 8.9 months. Early and delayed responders were characterized by a significant difference in depth of invasion beyond the mucosal layer.ConclusionsDelayed responders did not show differences in oncological outcomes compared with early responders. They were allowed to watch and wait for an additional 6 to 12 months without further treatment.

49. Primary low-grade B-cell lymphoma of the central nervous system: presentation and discussion of a case

49. Primary low-grade B-cell lymphoma of the central nervous system: presentation and discussion of a case

21 Primary hepatic low-grade B-cell lymphoma: A case report

21 Primary hepatic low-grade B-cell lymphoma: A case report

Clinical Epidemiology of Nonurothelial Bladder Cancer: Analysis of The Netherlands Cancer Registry

Nonurothelial malignancies represent a small fraction of bladder malignancies and are less extensively studied, resulting in sparse empirical data on these tumors. We sought insight into tumor characteristics and survival. Data were obtained from the nationwide Netherlands Cancer Registry on patient and tumor characteristics, and followup in all patients with primary invasive (T1 or greater) bladder tumors in The Netherlands between 1995 and 2006. Data were analyzed using frequency tables. Relative survival analysis was done. We identified 28,807 patients with invasive bladder cancer, of whom 7.7% presented with nonurothelial carcinoma. Mean patient age range at diagnosis of adenocarcinoma and soft tissue tumors was 66.4 years, and 78.3 years at diagnosis of nonspecified tumors. Most histological subtypes were more common in males except squamous cell carcinoma and lymphoma. Muscle invasion was seen in 52.2% of urothelial carcinoma cases vs 87.5%, 71.9% and 89.0% of squamous cell carcinoma, adenocarcinoma and neuroendocrine tumor cases, respectively. For urothelial carcinoma, squamous cell carcinoma and adenocarcinoma women presented at more advanced stage. In the neuroendocrine group this stage difference was the opposite. Survival analysis showed a 5-year relative survival rate of 32.2%, 22.9%, 31.8% and 21.1% for T2 or greater urothelial carcinoma, squamous cell carcinoma, adenocarcinoma and neuroendocrine tumors, respectively. Patients with nonurothelial carcinoma present at more advanced stage and overall have worse survival. Relative survival of muscle invasive adenocarcinoma equals survival of muscle invasive urothelial carcinoma. For stage II and III disease these cases do even better. Muscle invasive squamous cell carcinoma and neuroendocrine tumors show worse survival regardless of stage.

Juvenile onset of primary low-grade cutaneous B-cell lymphoma

Cutaneous lymphomas rarely occur in children and adolescents, and are mostly of the T-cell lineage. Low-grade primary cutaneous B-cell lymphoma (CBCL) is extremely rare in individuals under 18 years old. Only 11 patients under 20 years old have been reported in the literature. To evaluate the number of patients younger than 18 years with primary CBCL diagnosed at our centre and to investigate its clinicopathological features, treatment and course in this age group. We reviewed the files of all 90 patients with primary CBCL who attended the Department of Dermatology of our tertiary care university-affiliated centre from 1992 to 2007. Four patients who met study criteria were identified: three girls and one boy. Mean age at diagnosis was 16.6 years (range 16-17). Three patients had cutaneous marginal zone lymphoma (CMZL), and one had a spindle-cell (sarcomatoid) lymphoma, most probably follicular centre cell type. All were treated with the standard regimen used in adults. The mean duration of follow up was 45 months. No extracutaneous progression was noted. At present two of the four patients are in complete clinical remission. In Israel, primary CBCL apparently occurs more often in young patients than reported in the literature. CMZL is the most frequent type. Long follow up is mandatory to assess the biological behaviour of CBCL in the paediatric/adolescent age group.

Helicobacter pylori and MALT Lymphoma

Helicobacter pylori are gram-negative spiral microaerophilic organisms that belong to the Campylobacterales order, Helicobacteracea family. They are capable of colonizing the harsh environment of the human stomach. Over 50% of the world’s population carries this infection. 1 H pylori have colonized the human stomach since time immemorial. 2 In virtually all infected individuals, H pylori causes inflammation in the form of chronic active gastritis, which progresses in 10%–20% of affected persons to peptic ulcer disease, gastric adenocarcinoma, and/or mucosa-associated lymphoid tissue (MALT) lymphoma. However, only a very small minority (1%–2%) of infected individuals will develop a malignant disease. 3 One of the most distinctive features of H pylori is the genetic diversity it displays between clinical isolates. 4 H pylori isolates are highly diverse, with evidence of a constantly changing genome, primarily caused by point mutations, substitutions, insertions, and/or deletions. 5,6 Moreover, mixed infections are frequent and may lead to exchange of DNA fragments between H pylori strains in a single host. This suggests co-evolution of H pylori with its human host and that disease may be caused both by strain-specific properties that increase virulence and by host susceptibility. Such strains possibly evolve through exercising significant flexibility in gene content and gene regulation. Among many of the host–pathogen interactions that potentially could occur, some could prove beneficial in which the co-evolved bacteria and the host reach an almost symbiotic relationship. H pylori may not be just a bad bug in all instances. A large body of data have implicated H pylori in the pathogenesis of gastric MALT lymphoma, including epidemiologic, biological, and molecular genetic studies. Interestingly, both H pylori and MALT lymphoma were born almost simultaneously, when H pylori was described by Marshall and Warren 7 and MALT lymphoma was recognized as a distinct entity by Isaacson and Wright. 8 These latter investigators noted morphologic similarities between the histology of the condition known as immunoproliferative small intestinal disease, a subtype of primary intestinal B-cell lymphoma, and primary low-grade gastric B-cell lymphoma. Moreover, the histologic features differed from that of comparable nodal low-grade B-cell lymphomas in that the overall architecture and cytology of these lymphomas bore a resemblance to normal MALT tissue rather than recapitulating the structure of lymph nodes. Roughly one third of all non-Hodgkin’s lymphomas in adults are of extranodal origin. MALT lymphomas, by definition arising at mucosal/epithelial sites, collectively account for approximately 8% of all non-Hodgkin’s lymphomas, with gastric MALT lymphoma being the most common extranodal site. This review summarizes the published data that support a causative role for H pylori infection in the pathogenesis of gastric MALT lymphomas. The epidemiology of H pylori is discussed and its role in the pathogenesis, including factors affecting the infectious organism and the host. A major focus of this review addresses the relationship between the immune response, H pylori, and MALT lymphomas. This is followed by a description of the morphology, phenotype, and unifying concepts of the molecular genetics of MALT lymphomas. Treatment strategies of gastric MALT lymphoma are not discussed because these are beyond the scope of this review. Finally, we conclude with a brief discussion of H pylori–negative MALT lymphomas, preventative measures for reducing infection, and the direction of future research into MALT lymphomas.

Primary low‐grade B‐cell lymphoma of the endometrium

Primary low‐grade B‐cell lymphoma of the endometrium

Primary low-grade B-cell lymphoma of mucosa-associated lymphoid tissue of the dura mimicking the presentation of an acute subdural hematoma

The authors present the case of a 64-year-old woman who experienced a left hemiparesis. An initial diagnosis of subdural hematoma was made based on results of computerized tomography scanning. Subsequent magnetic resonance imaging indicated an extraaxial meningioma. Histological findings confirmed an extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT). The authors outline the natural history of central nervous system lymphomas and of MALT lymphomas in other tissues. They review seven previously reported cases and emphasize the importance of recognizing these tumors as a distinct clinicopathological entity.

A Case of Primary Pulmonary Lymphoma of Bronchus-Associated Lymphoid Tissue associated with Systemic Lupus Erythematous

A primary pulmonary malignant lymphoma is a rare disease. It is thought to be a category of non-Hodgkin's lymphoma arising from the bronchous-associated lymphoid tissue (BALT). The majority of primary pulmonary lymphomas are low-grade, small B-cell lymphomas, which are associated with Sjogren's syndrome and similar autoimmune disorders. A case of primary pulmonary low-grade B-cell lymphoma arising from the BALT was encountered in a patient with systemic lupus erythematosus. A 54-year-old man was admitted to the hospital for the evaluation of left pleuritic chest pain and multiple joint pain in both hands. Serologic tests for collagen vascular disease were performed. The results of ANA and anti-ds-DNA were all positive. The computed tomography of the chest showed patchy consolidations in the left lower lobe with a pleural effusion and a video-assisted thoracoscopic biopsy was performed. Here we report a case of a low-grade B-cell lymphoma of BALT in a patient with systemic lupus erythematosus with a review of the relevant literatures.

Primary Low-grade B-cell Lymphoma of Mucosa-associated Lymphoid Tissue (MALT)-type of the Liver in a Patient with Hepatitis C Virus Infection

We report a case of primary low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT)-type of the liver presenting as a solitary mass in a patient with hepatitis C virus infection. A 73-year-old male was referred to our hospital because of a solitary mass in the liver, which was identified by ultrasonography during a routine follow-up study for his hepatitis C virus carrier status. A fine-needle biopsy was performed and the mass was diagnosed as non-Hodgkin's lymphoma. A detailed investigation showed no evidence of lymphoma in other sites. Left lateral segmentectomy was performed. Histologic examination showed diffuse, monotonous infiltration of centrocyte-like cells that are CD 19(+), CD 20(+), sIgM- (+), CD 3 (m ), CD 5 (m ), CD 10 (m ). The patient received no adjuvant chemotherapy and is alive and disease free 34 months after surgery.

Primary low‐grade MALT lymphoma of the gallbladder

We report a case of primary low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue (low-grade MALT lymphoma) in the gallbladder. A 58-year-old woman suspected of gallbladder carcinoma underwent laparoscopic cholecystectomy. Microscopic examination of the gallbladder demonstrated lymphoid cell infiltration forming lymphoid follicles with hyperplastic secondary follicles. The surrounding monocytoid B cells and centrocyte-like cells selectively infiltrated the crypt epithelium forming lympho-epithelial lesions. Plasma cells were also noted beneath the mucosal epithelium. Bile culture revealed the Gram-negative bacilli Enterococcus faecalis and Morganella morganii. Immunoglobulin heavy chain gene rearrangement was confirmed using polymerase chain reaction (PCR) and oligoclonal lymphoid proliferations was detected. Because autoimmune diseases, or chronic inflammatory disorders, seem to correlate with the occurrence of MALT lymphoma, Gram-negative bacterial infection could also be considered as a prodrome of MALT lymphoma of the gallbladder.

Conservative treatment of primary gastric low-grade B-cell lymphoma of mucosa-associated lymphoid tissue: Predictive factors of response and outcome

Conservative treatment of primary gastric low-grade B-cell lymphoma of mucosa-associated lymphoid tissue: predictive factors of response and outcome

Conservative treatment of primary gastric low-grade B-cell lymphoma of mucosa-associated lymphoid tissue: Predictive factors of response and outcome

Conservative treatment of primary gastric low-grade B-cell lymphoma of mucosa-associated lymphoid tissue: Predictive factors of response and outcome

Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma of the Thymus

We present a rare case of primary low-grade B-cell lymphoma of the thymus. The tumor showed the typical features of a MALT (mucosa-associated lymphoid tissue) lymphoma with epithelium-lined thymic cyst. Histologically, there were sheets of centrocyte-like (CCL) cells intermingled with small lymphocytes and plasma cells. CCL cells invaded the epithelium of the cyst and residual Hassall's corpuscles, forming characteristic lymphoepithelial lesions. Immunohistochemically, the tumor cells revealed positive immunoreaction for CD20 and monotypic cytoplasmic restriction of kappa light chain.

A Case of Primary Pulmonary Low-grade B-cell Lymphoma of Bronchus-associated lymphoid tissue with Bilateral Consolidation and Indolent Clinical Course

Primary pulmonary lymphoma (PPL) is an uncommon tumor, which constitutes 3-4% of all extranodal lymphomas and 0.3-0.5 % of all primary pulmonary malignant tumors. Low-grade B-cell lymphomas of bronchus-associated lymphoid tissue (BALT) accounted for the majority of PPL. This BALT lymphomas are frequently asymptomatic and have an excellent prognosis and an indolent clinical course by contrast with T-cell type. Therefore, determination of the B- or T-immunophenotype of the tumor cells is known to be very important from a clinical aspect. Recent advances in immunohistochemical techniques, cytogenetics, and molecular biology have allowed better definition of type, maturation, and clonality of lymphoma cells and have made it possible to better understand the PPL. We experienced an asymptomatic 43-year-old man who was evaluated for infiltrates on both sides discovered incidentallly after a routine chest roentgenogram. He was eventually diagnosed as low-grade B-cell lymphoma of BALT by immunohistochemical staining from specimens obtained by open lung biopsy. He was treated with combination chemotherapy. At follow up 12 mons following initial diagnosis, he remains in stable. We report this case, who showed a relatively favorable prognosis and indolent clinical course compatible with low-grade B-cell lymphoma.

Primary low-grade B-cell lymphoma of MALT-type occurring in the liver: a study of two cases

Background/Methods: Primary non-Hodgkin's lymphomas of the liver are rare. One specific clinico-pathological entity has been identified as hepatosplenic γ/δ T-cell lymphoma. Recently, another distinct primary lymphoma of the liver has been recognised as primary low-grade hepatic B-cell lymphoma of mucosa-associated lymphoid tissue (MALT), based on a study comprising four cases. We analysed two additional cases of this particular non-Hodgkin's lymphoma, not only by morphology and phenotyping, but also by genotyping and cytogenetic analysis. Results: This type of non-Hodgkin's lymphoma is characterised by a dense lymphoid infiltrate, localised in the portal tracts, and is associated with lympho-epithelial lesions of the bile ducts, thereby mimicking hepatitis or an inflammatory bile duct disorder. In one of our cases, translocation t(3;14)(q27;q32) was identified as the sole cytogenetic abnormality. A high incidence of trisomy 3 has been associated with marginal zone B-cell lymphomas, and fluorescence in situ hybridisation as well as comparative genomic hybridisation studies have shown frequent involvement of the long arm of chromosome 3. Nevertheless, t(3;14)(q27;q32) involving BCL6 gene, located at 3q27, has not yet been found. Conclusion: Our findings suggest a role for the BCL6 gene in the histogenesis of this particular lymphoma.

Primary low-grade B-cell lymphoma of the dura: a mucosa associated lymphoid tissue-type lymphoma.

The clinicopathologic findings in five patients with primary low-grade B-cell lymphoma of the intracranial dura are described. All patients were women, 40-62 years of age, who presented with focal neurologic symptoms. Radiologic studies showed a well-localized dural mass in each case, raising a preoperative diagnosis of meningioma. Cytologically these were composed of a diffuse infiltrate of small lymphocytes with plasmacytoid differentiation, with a variable admixture of centrocytelike cells. Lambda light chain restriction was found in three cases, and kappa light chain in one. VJ polymerase chain reaction for immunoglobulin heavy-chain rearrangement was positive in three of four cases, including one case in which immunostaining results were equivocal. Staging procedures did not show involvement at any other site. Therapy consisted of radiation (n = 3), chemotherapy (n = 1), or both (n = 1), with excellent response. There was no evidence of recurrence or subsequent dissemination at follow-up of up to 63 months. Low-grade B-cell lymphomas arising in the intracranial dura are rare but appear to be similar to other low-grade B-cell lymphomas arising in extranodal sites in terms of clinical presentation as stage 1E disease, indolent behavior, and favorable response to treatment, suggesting that they may be part of the mucosa associated lymphoid tissue (MALT) lymphoma spectrum. They appear to arise at dural sites where meningothelial cells are concentrated.

Low-Grade B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue Arising in the Thymus

We describe two cases of primary low-grade B-cell lymphoma of the thymus that showed histological features of low-grade B-cell lymphoma arising in mucosa-associated lymphoid tissue (MALT). The appearances most closely resembled MALT lymphoma arising in myoepithelial sialadenitis (MESA). In both cases, the tumor was excised. In one case, there has been no recurrence in 4 years of follow-up without further treatment; in the second case, the tumor has involved an axillary lymph node. Immunohistochemistry showed light-chain restriction in both cases, and the B-cell phenotype was similar to that previously described in MALT lymphomas. The occurrence of MALT lymphoma in the thymus is consistent with the presence of mucosal structures (Hassall's corpuscles) and with recent descriptions of a native B-cell population in this organ. The relationship of this previously undescribed thymic low-grade B-cell MALT lymphoma arising in the thymus has not yet been clarified.