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Long-standing thyroid swellings can present therapeutic and diagnostic challenges. Although benign, these nodules can harbour malignancies of diverse lineages ranging from those of follicular cell origin to rare malignancies such as lymphomas and paragangliomas. Here, the authors are sharing their three clinical experiences with thyroid neoplasms of long duration. The first case is a 42-year-old male patient who presented with an anterior neck swelling of 12 years' duration. Postoperatively, a diagnosis of Mucosa-associated Lymphoid Tissue (MALT) lymphoma was made based on histopathology and immunohistochemistry. In the second case, a 43-yearold male presented with a neck swelling with eight years of history. Intraoperatively, the lesion was found to be locally aggressive with infiltration into the strap muscles and trachea. A tissue diagnosis of paraganglioma of the thyroid gland was made postoperatively. The third case involves a 72-year-old female who had undergone a left thyroidectomy 10 years back and presented to the Outpatient Department (OPD) with a history of right-sided neck swelling associated with dysphagia and a change in voice. The patient was diagnosed with anaplastic carcinoma, specifically squamous cell carcinoma. Thyroid lesions of abnormally large size create intraoperative problems concerning securing an adequate airway, locating anatomical structures, especially the Recurrent Laryngeal Nerve (RLN), parathyroids, and great vessels of the neck. Postoperatively, the tissue features may be very uncharacteristic, making reaching a proper diagnosis difficult. The use of immunohistochemistry helps in determining the cell lineage and type of malignancy. [ABSTRACT FROM AUTHOR]

Long-standing thyroid swellings can present therapeutic and diagnostic challenges. Although benign, these nodules can harbour malignancies of diverse lineages ranging from those of follicular cell origin to rare malignancies such as lymphomas and paragangliomas. Here, the authors are sharing their three clinical experiences with thyroid neoplasms of long duration. The first case is a 42-year-old male patient who presented with an anterior neck swelling of 12 years’ duration. Postoperatively, a diagnosis of Mucosa-associated Lymphoid Tissue (MALT) lymphoma was made based on histopathology and immunohistochemistry. In the second case, a 43-year-old male presented with a neck swelling with eight years of history. Intraoperatively, the lesion was found to be locally aggressive with infiltration into the strap muscles and trachea. A tissue diagnosis of paraganglioma of the thyroid gland was made postoperatively. The third case involves a 72-year-old female who had undergone a left thyroidectomy 10 years back and presented to the Outpatient Department (OPD) with a history of right-sided neck swelling associated with dysphagia and a change in voice. The patient was diagnosed with anaplastic carcinoma, specifically squamous cell carcinoma. Thyroid lesions of abnormally large size create intraoperative problems concerning securing an adequate airway, locating anatomical structures, especially the Recurrent Laryngeal Nerve (RLN), parathyroids, and great vessels of the neck. Postoperatively, the tissue features may be very uncharacteristic, making reaching a proper diagnosis difficult. The use of immunohistochemistry helps in determining the cell lineage and type of malignancy.

Indolent lymphomas of the gastrointestinal (GI) tract represent a challenging diagnostic area. Around one third of extranodal non-Hodgkin lymphomas (NHL) involve the GI tract. This may be primary, including several relatively recently described entities such as duodenal-type follicular lymphoma and intestinal T-cell lymphoma of the GI tract, or secondary involvement by systemic lymphoma. As a significant proportion of lymphomas may initially present in the GI tract, associated with non-specific GI symptoms without strong clinical suspicion for haematological malignancy, it is crucial that general and gastrointestinal pathologists are familiar with these entities. The differential diagnosis for an indolent-appearing lymphoid infiltrate in the GI tract ranges from reactive or benign conditions such as nodular follicular hyperplasia or coeliac disease, to highly aggressive lymphomas such as monomorphic epitheliotropic T-cell lymphoma. The aim of this review is to discuss indolent B-, T- and NK-cell lymphomas of the GI tract including the classification of the more recently described entities, with an emphasis on morphological and immunohistochemical features that may help the pathologist differentiate these from benign or aggressive malignant mimics, having significant implications for management and prognosis. [ABSTRACT FROM AUTHOR]

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Although gastric mucosa-associated lymphoid tissue (MALT) lymphoma without Helicobacter pylori (HP) has increased recently, a specific endoscopic classification has not been established; its endoscopic characteristics have not been investigated. In this study, we retrospectively investigated gastric MALT lymphoma without HP in our hospital and assessed differences in the endoscopic findings according to HP infection status. Fifty-seven patients with gastric MALT lymphoma Lugano stage I, diagnosed between January 2013 and March 2023, were divided into three groups (currently HP infected, previously infected, and uninfected), wherein their endoscopic findings were evaluated. Furthermore, the superficial type, as per the classification of Sano et al., was independently subdivided based on the endoscopic differential diagnoses, as follows: atrophic gastritis-like, angiodysplasia-like, superficial gastritis-like, and undifferentiated carcinoma-like. Compared with the currently infected group, the HP-uninfected group tended to have more small lesions without erosion and more discolored, undifferentiated carcinoma-like depressed lesions. In addition, the positive rate of the tree-like appearance (TLA) and ballooning characteristics of gastric MALT lymphoma in magnified findings was lower in the HP-uninfected group. In patients without HP infection, MALT lymphoma should be excluded, even in the absence of suspicious magnifying findings such as TLA or ballooning. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES The goal of this project was to evaluate the effect of surgical treatment and the long-term survival of patients with staged IE/IIE pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma. METHODS From January 2004 to December 2018, we retrospectively analysed 96 patients diagnosed with low-stage primary pulmonary MALT lymphoma according to the modified Ann Arbor staging system (IE/IIE). We compared the outcomes of different treatment modalities for staged IE/IIE MALT lymphoma. Progression-free survival (PFS) and overall survival were estimated using Kaplan-Meier curves, and the differences were compared using the log-rank test. The Cox proportional hazards model was used in this study. RESULTS The median PFS time of low-staged MALT lymphomas was 118 months. The overall survival and PFS of the radical surgery group and the biopsy + chemotherapy group suggested no significant difference (P  =   0.63, P  =   0.65). Patients positive for Blc-2 and Ki-67 suffered from a compromised PFS (P  =   0.023, P  =   0.006). The Cox adjusted proportional hazards model analysis suggested that surgical procedures were not protective factors for patients with low-staged (IE/IIE) pulmonary MALT lymphoma, whereas being positive for Blc-2 and Ki-67 was a risk factor for patients with low-staged pulmonary MALT lymphoma (hazard ratio: 9.567; P =  0.044; hazard ratio: 6.042, P =  0.049). CONCLUSIONS Our findings suggested that for staged IE/IIE pulmonary MALT lymphoma, radical surgical resection did not provide a survival benefit compared with chemotherapy after biopsy. Thus, radical surgery may be avoided unless biopsy is necessary for a diagnosis that requires sublobar resection. For those lesions that were Blc-2- or Ki-67-positive, compromised survival may be suggested. [ABSTRACT FROM AUTHOR]

This document is a guideline published in the British Journal of Haematology that provides healthcare professionals with clear guidance on the diagnosis and management of patients with marginal zone lymphoma (MZL). MZL is a group of clinically indolent mature B-cell lymphomas that arise from memory B cells in the marginal zones of secondary lymphoid tissues. The guideline discusses the different subtypes of MZL, diagnostic methods, and potential aetiological factors. It also includes information on treatment options for different stages of MZL, including radiotherapy, systemic therapy, and targeted agents. The document emphasizes the importance of individualized patient care and shared decision making. [Extracted from the article]

Objectives Gastric mucosa-associated lymphoid tissue (MALT) lymphoma is historically associated with Helicobacter pylori (HP) infections in more than 80% of patients. However, the incidence of HP-negative MALT lymphoma has been increasing. The clinicopathologic features have not been well studied, and optimal management strategies remain unclear. Methods The pathology database was searched for primary gastric MALT lymphomas diagnosed from 2000 to 2017. The clinical data and the slides were reviewed. The cases were divided for analysis into those with a background of chronic gastritis with HP, chronic gastritis without HP, and without either a background of chronic gastritis or HP. Results Of 70 gastric MALT lymphoma cases identified, 26 (37% of total) had chronic gastritis and were positive for HP histologically (n = 23) or were HP positive by additional laboratory testing (n = 3). The remaining 44 (63% of total) cases were HP negative by histology. Within the HP-negative cases, 5 (11% of HP-negative cases) showed histologic gastritis while 39 (89% of HP-negative cases) did not have sufficient evidence of gastritis through review of slides (n = 18) or based on available pathology reports (n = 21). The HP-negative cases without gastritis had higher propensities to show a mass lesion on endoscopy compared with HP-positive cases (37.5% vs 11.1%, P =.02) at the initial diagnosis. The immunophenotype and rate of positive B-cell gene rearrangement were not significantly different between the 2 groups. While all HP-positive patients received antibiotics for HP eradication, treatment in the HP-negative group varied among antibiotics, radiation, rituximab, or chemotherapy. Among HP-negative patients with available follow-up, 13 (39%) showed disease recurrence, similar to the recurrence rate in HP-positive patients; however, no individual from either group has died of the disease thus far. Conclusions The incidence of HP-negative MALT lymphoma is increasing, and in our practice, it is currently more common than HP-associated MALT lymphomas. The pathophysiology of HP-negative MALT lymphoma without chronic gastritis remains unclear. Follow-up data in our study suggest that the prognosis of these cases is excellent despite varied management modalities. [ABSTRACT FROM AUTHOR]

Inducing humoral and cytotoxic mucosal immunity at the sites of pathogen entry has the potential to prevent the infection from getting established. This is different from systemic vaccination, which protects against the development of systemic symptoms. The field of mucosal vaccination has seen fewer technological advances compared to nucleic acid and subunit vaccine advances for injectable vaccine platforms. The advent of the next-generation adenoviral vectors has given a boost to mucosal vaccine research. Basic research into the mechanisms regulating innate and adaptive mucosal immunity and the discovery of effective and safe mucosal vaccine adjuvants will continue to improve mucosal vaccine design. The results from clinical trials of inhaled COVID-19 vaccines demonstrate their ability to induce the proliferation of cytotoxic T cells and the production of secreted IgA and IgG antibodies locally, unlike intramuscular vaccinations. However, these mucosal vaccines induce systemic immune responses at par with systemic vaccinations. This review summarizes the function of the respiratory mucosa-associated lymphoid tissue and the advantages that the adenoviral vectors provide as inhaled vaccine platforms. [ABSTRACT FROM AUTHOR]

With the increasing opportunities for medical management of von Hippel–Lindau (VHL)-related lesions, fundamental questions are arising regarding the possibilities of the FDA-approved medication (Belzutifan) to delay the progression of other non-VHL-related tumors that are not requiring urgent surgical interventions. We present a follow-up report on a VHL patient, with a co-morbid history of pulmonary mucosa-associated lymphoid tissue lymphoma, who was initially described in the clinical scientific literature ~10 years ago. This patient's case highlights some of the challenges for some VHL patients with comorbid non-VHL-related tumors and potential opportunities for targeted support. [ABSTRACT FROM AUTHOR]

Although gastric mucosa-associated lymphoid tissue (MALT) lymphoma without Helicobacter pylori (HP) has increased recently, a specific endoscopic classification has not been established; its endoscopic characteristics have not been investigated. In this study, we retrospectively investigated gastric MALT lymphoma without HP in our hospital and assessed differences in the endoscopic findings according to HP infection status. Fifty-seven patients with gastric MALT lymphoma Lugano stage I, diagnosed between January 2013 and March 2023, were divided into three groups (currently HP infected, previously infected, and uninfected), wherein their endoscopic findings were evaluated. Furthermore, the superficial type, as per the classification of Sano et al., was independently subdivided based on the endoscopic differential diagnoses, as follows: atrophic gastritis-like, angiodysplasia-like, superficial gastritis-like, and undifferentiated carcinoma-like. Compared with the currently infected group, the HP-uninfected group tended to have more small lesions without erosion and more discolored, undifferentiated carcinoma-like depressed lesions. In addition, the positive rate of the tree-like appearance (TLA) and ballooning characteristics of gastric MALT lymphoma in magnified findings was lower in the HP-uninfected group. In patients without HP infection, MALT lymphoma should be excluded, even in the absence of suspicious magnifying findings such as TLA or ballooning.

We report a case of an 88-year-old female who presented to us with dysphagia, change in voice, and stridor, the diagnosis of which turned out to be primary laryngeal lymphoma. It is a rare condition, and repeated biopsies are needed in most of the cases. It has a very good response to radiotherapy and chemotherapy. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the ultrasonographic features in patients with primary uveal mucosa-associated lymphoid tissue (MALT) lymphoma. Methods: Medical records of 12 patients (13 eyes) diagnosed with primary uveal MALT lymphoma between September 2014 and September 2021 were retrospectively reviewed. Ultrasonography, B-scan ultrasonography, color Doppler flow imaging, and ultrasound biomicroscopy findings were retrieved from the medical records. Results: Mean age of the included patients was 59.4 ± 8.6 years. Typical ultrasonographic features of the choroidal infiltrates were flat, diffuse, and thickened, with low and homogenous internal reflectivity and with rich arterial blood flow from posterior ciliary arterioles. The mean thickness of the choroidal infiltrates was 1.34 ± 0.68 mm (n = 13). Most of the affected eyes had posterior episcleral extensions, with a mean thickness of 1.66 ± 1.21 mm (n = 12). Typical crescent-like posterior episcleral extensions were detected in nine eyes (69.2%). In six eyes, the blood flow from the choroidal infiltrates communicated with the episcleral extensions. In the ciliary body, the mean thickness of the infiltrates was 1.08 ± 0.43 mm (n = 9), and seven eyes (77.8%) had 360° ring-like infiltrations. The initial best-corrected visual acuity (BCVA) was significantly correlated with the final BCVA after treatment (p < 0.001). Conclusion: Multipurpose ultrasonographic imaging revealed the unique characteristics of the primary uveal MALT lymphoma and is helpful in the diagnosis of this rare disease. [ABSTRACT FROM AUTHOR]

Abstract Between January 2010 and December 2015, we enrolled 28 patients with stage IEI/IIE1 extragastric mucosa‐associated lymphoid tissue (MALT) lymphoma who received first‐line antibiotic treatment, after informing them about the pros and cons of alternative therapies. In addition, during the same period, 64 patients with stage IE/IIE1 disease who received conventional treatment were selected as the control group. The most common primary sites were the ocular adnexal area (17 cases), followed by the salivary glands (four cases), pulmonary (three cases), and thyroid, trachea, larynx, and colon region (one case each). First‐line antibiotic treatment resulted in an overall response rate of 57.1%: 12 patients achieved complete remission (CR), while four achieved partial remission (antibiotic‐responsive tumors). Monoclonal gammopathy was significantly prevalent in antibiotic‐unresponsive tumors than in antibiotic‐responsive tumors (50.0% [6/12] vs. 12.5% [2/16], p = 0.044). After a median follow‐up of 7 years, all patients with CR remained lymphoma‐free, with 7‐year event‐free survival (EFS) and overall survival (OS) rates of 62.7% and 96.4%, respectively. The 7‐year EFS and OS rates of patients who received conventional treatments were 73.1% and 91.1%, respectively. Compared with that noted in patients who received conventional treatment, antibiotic treatment was effective in some patients with localized extragastric MALT lymphoma.

Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis worldwide, with varied clinical and histopathological features between individuals, particularly across races. As an autoimmune disease, IgAN arises from consequences of increased circulating levels of galactose-deficient IgA1 and mesangial deposition of IgA-containing immune complexes, which are recognized as key events in the widely accepted "multi-hit" pathogenesis of IgAN. The emerging evidence further provides insights into the role of genes, environment, mucosal immunity and complement system. These developments are paralleled by the increasing availability of diagnostic tools, potential biomarkers and therapeutic agents. In this review, we summarize current evidence and outline novel findings in the prognosis, clinical trials and translational research from the updated perspectives of IgAN pathogenesis. [ABSTRACT FROM AUTHOR]

Primary intracranial mucosa-associated lymphoid tissue (MALT) lymphoma is a rare type of brain tumor, with only a few reported cases worldwide that mostly have only one lesion with conventional magnetic resonance imaging (MRI) findings. Here, we present a special case of intracranial MALT lymphoma with two mass lesions radiographically consistent with meningiomas on MRI before the operation. A 66-year-old woman was admitted to the hospital with intermittent right facial pain for 1 year, aggravated for the last month. Brain MRI showed two extracerebral solid masses with similar MR signal intensity. One mass was crescent-shaped beneath the skull, and the other was in the cavernous sinus area. Lesions showed isointensity on T1WI and T2WI and an intense homogeneous enhancement after contrast agent injection. Both lesions showed hyperintensity in amide proton transfer-weighted images. The two masses were all surgically resected. The postoperative pathology indicated extranodal marginal zone B-cell lymphoma of MALT. To improve awareness of intracranial MALT lymphoma in the differential diagnosis of extraaxial lesions among clinicians, we present this report and briefly summarize previously reported cases to describe the clinical, pathological, radiological, and treatment features. [ABSTRACT FROM AUTHOR]

Simple Summary: Mucosa-associated lymphoid tissue is a diffuse system of aggregations of lymphoid tissues found in the gut, nasopharynx, bronchus, tonsils, eye, skin, salivary glands, and urinary tract, which are in close contact with the outer environment. We previously reported the presence of lymphoid tissue in the mucosa of the vaginal vestibule in cows, but information regarding other animals is scarce. This study investigated the gross anatomical and histological features of lymphoid tissue in the mucosa of the vaginal vestibule of goats and pigs. The vaginal vestibules of healthy adult goats and pigs were examined using hematoxylin and eosin staining, immunohistochemistry, and immunofluorescence. Furthermore, scanning electron microscopy was used for analyzing the ultrastructure of the mucosal surface. Goat and pig lymphoid tissues comprised B cells, T cells, macrophages, dendritic cells, and plasma cells. Furthermore, the vaginal vestibule epithelium covering lymphoid tissues was disrupted by a large intercellular space in both goats and pigs, and immune cells directly contacted the luminal space through this area lacking epithelium. No obvious differences were found between goats and pigs, suggesting that genital organ-associated lymphoid tissue is a conserved local immunological barrier in these animals. Mucosa-associated lymphoid tissue (MALT) is a specialized form of peripheral lymphoid tissue (LT), which is found on mucosal surfaces exposed to the environment. However, morphological data of these tissues in farm animals are scarce. This study investigated the gross anatomical and histological features of genital organ-associated lymphoid tissues (GOALTs) in the vaginal vestibule (VV) of healthy, non-pregnant, adult goats and pigs. Their VVs were composed of stratified squamous, non-keratinized epithelium, and various-sized dark-blue hematoxylin-positive spots were observed in whole-mount specimens, which were diffusely distributed throughout the mucosal surfaces. These spots were histologically identified as LTs and consisted of lymphatic nodules (LNs) or diffuse lymphoid tissue (DLTs). Both LNs and DLTs contained B cells, T cells, macrophages, dendritic cells, plasma cells, and high endothelial venules. Only the numbers of B cells were significantly higher in both the LNs and DLTs of pigs compared to goats. Furthermore, the surface of the VV epithelium covering the LTs was partially disrupted with a large intercellular space containing abundant connective tissue fibers with numerous lymphocytes. In conclusion, GOALTs in the VV appear to be common local immunological barriers in both examined animals. This knowledge is crucial for understanding the structures and disorders of female reproductive organs in farm animals. [ABSTRACT FROM AUTHOR]

We report an atypical case of diffuse large B-cell lymphoma (DLBCL) of bilateral lacrimal glands and paranasal sinus following mucosa-associated lymphoid tissue (MALT) lymphoma of the lung. Bilateral DLBCL is rare in the literature, and only few cases of DLBCL in bilateral lacrimal gland are reported. A 71-year-old male presented with bilateral, slowly enlarging, and swelling of both eyelids. Computed tomography scan images showed bilateral symmetric, hyperdense, circumferential masses over lacrimal glands occupying most of the orbital compartment. Neither optic nerve involvement nor adjacent orbital walls erosion was noted. Bilateral excisional biopsy and pathological examination confirmed the diagnosis of DLBCL. Since DLBCL of bilateral lacrimal gland can occur in case of systemic MALT lymphoma, excision and pathological examination is mandatory, and further metastatic workup is essential.

Primary intracranial mucosa-associated lymphoid tissue (MALT) lymphoma is a rare type of brain tumor, with only a few reported cases worldwide that mostly have only one lesion with conventional magnetic resonance imaging (MRI) findings. Here, we present a special case of intracranial MALT lymphoma with two mass lesions radiographically consistent with meningiomas on MRI before the operation. A 66-year-old woman was admitted to the hospital with intermittent right facial pain for 1 year, aggravated for the last month. Brain MRI showed two extracerebral solid masses with similar MR signal intensity. One mass was crescent-shaped beneath the skull, and the other was in the cavernous sinus area. Lesions showed isointensity on T1WI and T2WI and an intense homogeneous enhancement after contrast agent injection. Both lesions showed hyperintensity in amide proton transfer–weighted images. The two masses were all surgically resected. The postoperative pathology indicated extranodal marginal zone B-cell lymphoma of MALT. To improve awareness of intracranial MALT lymphoma in the differential diagnosis of extra-axial lesions among clinicians, we present this report and briefly summarize previously reported cases to describe the clinical, pathological, radiological, and treatment features.

The mammalian nasopharynx is an anatomically complex region of the upper respiratory tract that directly communicates with the nasal cavity, laryngopharynx, oesophagus and trachea. The nasopharyngeal mucosa contains moderate quantities of mucosa-associated lymphoid tissue (MALT) that is appropriately located for immunological sampling but also creates vulnerability to pathogens. In recent years, the nasopharynx has been inculpated in the pathogenesis of important diseases of cattle (foot-and-mouth disease) and humans (COVID-19), yet the tissue has never been described in detail in any species. In order to characterize the morphology and cellular composition of the bovine nasopharynx, samples of mucosa were collected from the nasopharynx of five 8–13-month-old steers and examined using light microscopy, immunohistochemistry and multichannel immunofluorescence. Morphologically, the nasopharyngeal epithelium was highly heterogeneous, with a continuum ranging from stratified squamous epithelium to highly attenuated, follicle-associated epithelium (FAE). Distribution of MALT was similarly regionally variable ranging from absent to clusters of multiple lymphoid follicles. Phenotypic characterization demonstrated dense distributions of dendritic cells and T lymphocytes surrounding lymphoid follicles, which comprised mostly B lymphocytes. The FAE overlaying the lymphoid follicles also contained higher numbers of dendritic cells and lymphocytes compared with the adjacent non-lymphoid epithelium, although cytotoxic T cells were notably scarce in the FAE. The bovine nasopharyngeal lymphoid tissue had comparable elements to other MALTs with specific differences that may help to elucidate the pathogenesis of infectious agents that have specific tropism for this tissue. [ABSTRACT FROM AUTHOR]

Purpose: It is well known that the majority of the extranodal marginal zone lymphomas of mucosa-associated lymphoid tissues (MALT lymphomas) are associated with microbiota, e.g., gastric MALT lymphoma with Helicobacter pylori. In general, they are very sensitive to low-dose radiotherapy and chemotherapeutic agents. The microbiota profile is not clearly elucidated in bronchus-associated lymphoid tissue (BALT) lymphoma, a rare type of MALT lymphoma in the lung. Thus, this study aimed to clarify the intratumor microbiome in BALT lymphoma using the third-generation NGS method., Materials and Methods: DNAs were extracted from 12 formalin-fixed paraffin-embedded (FFPE) tumor tissues obtained from BALT lymphoma patients diagnosed between 1990 and 2016. 16S rRNA gene was amplified by polymerase chain reaction. Amplicons were sequenced using a Nanopore platform. Next-generation sequencing analysis was performed to assess microbial profiles. For comparison, FFPE specimens from nine non-cancerous lung tissues were also analyzed., Results: Specific bacterial families including Burkholderiaceae, Bacillaceae, and Microbacteriaceae were associated with BALT lymphoma by a linear discriminant analysis effect size approach. Although the number of specimens was limited, BALT lymphomas exhibited significantly higher microbial abundance and diversity with distinct microbial composition patterns and correlation networks than non-cancerous lung tissues., Conclusion: This study provides the first insight into intratumor microbiome in BALT lymphoma using the third-generation NGS method. A distinct microbial composition suggests the presence of a unique tumor microenvironment of BALT lymphoma.

Liping Xu,&ast; Xinyu Tang,&ast; Nan Jiang,&ast; Sheng Zhang, Yuandong Cao, Xinchen Sun Department of Radiation Oncology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu Province, People’s Republic of China&ast;These authors contributed equally to this workCorrespondence: Xinchen Sun; Yuandong CaoDepartment of Radiation Oncology, The First Affiliated Hospital of Nanjing Medical University, No. 300 Guangzhou Road, Nanjing, Jiangsu Province, 210029, People’s Republic of ChinaEmail sunxinchen2012@163.com; yuandongcao@163.comPurpose: Lymphomas of the orbit and ocular adnexa are histologically heterogeneous and their incidence rate has been increasing lately. However, because of their rarity and diversity, few cases have been analyzed. This study evaluated patients with orbital and ocular adnexal mucosa-associated lymphoid tissue (OAMALT), who received radiotherapy, and categorized their clinical characteristics, treatment outcomes, and complications.Patients and Methods: We collected data on clinical presentation, age, sex, imaging, tumor location, treatment methods, pathological diagnosis, cataract incidence, cataract incidence periods, overall survival (OS), and disease-free survival (DFS) from 32 patients with orbital involvement and pathologically confirmed marginal zone B-cell lymphoma of MALT who were treated between 2009 and 2018. Twenty-two patients received 20 Gy/10 Fr using intensity-modulated radiation therapy (IMRT) plus 14 Gy/7 Fr using a 6– 14-MeV electron beam therapy using a lens-sparing approach. Ten patients received 32 Gy/16 Fr or 34 Gy/17 Fr using IMRT without the lens shield technique. Kaplan–Meier analysis was used to estimate DFS and OS.Results: The median follow-up time was 83.4 ± 24.5 months. No patient had local recurrence, although three patients developed distant metastases. The 5-year and 10-year OS rates were both 100%. The 5-year and 10-year DFS rates were 96.7% and 74.2%, respectively. Overall, 11 (32.4%) of the 34 lenses developed cataracts. The estimated 5-year, 7-year, and 10-year cumulative cataract rates were 6.9%, 30.9%, and 60.8%, respectively. The median cumulative cataract incidence period was 107.0 months. Age was the only significant parameter associated with cataract formation.Conclusion: A radiation dose of 32– 34 Gy yields excellent local control, DFS, and OS for OAMALT. Some patients may have systemic relapse, and better identification of these patients is necessary. Reducing the prescription radiation dose or using better radiation techniques to spare the ipsilateral lens could reduce cataract formation.Keywords: orbital lymphoma, adnexal lymphoma, mucosa-associated lymphoid tissue, radiotherapy

Background: To date, there is no treatment consensus on mucosa-associated lymphoid tissue (MALT) derived primary pulmonary lymphoma (PPL). Methods: We identified patients with early-stage MALT-type PPL from the National Cancer Institute's Surveillance, Epidemiology, and End Results program database. The patients were divided into four groups according to treatment modalities: None of surgery or chemotherapy (None) group, Surgery alone group, Chemotherapy alone (Chemo alone) group, and Surgery plus chemotherapy (Surgery + chemo) group. Overall survival (OS) and cancerspecific survival (CSS) were study endpoints. We performed Cox regression analyses, propensity score-matched analyses (PSM) and Kaplan-Meier (KM) survival curves to compare the survival among different groups. Results: A total of 953 patients were included in our analysis with 302, 403, 175, and 73 cases in the None, Surgery alone, Chemo alone, and Surgery + chemo groups, respectively. In this cohort, the estimated 3-year, 5-year and 10-year OS rates were 86.95%, 78.91%, and 55.89%, respectively. Meanwhile, the estimated 3-year, 5-year and 10-year CSS rates were 96.71%, 93.73%, and 86.84%, respectively. Multivariate Cox regression analyses demonstrated that increasing age, tumors located in the lower lobe, and stage II were significant predictors of poorer OS while increasing age and tumors located in the bilateral lungs were associated with lower CSS. After PSM analyses, the KM survival curves showed no significant differences in OS or CSS among the four groups. Conclusion: Early-stage MALT-type PPL is indolent in nature. Neither surgery, chemotherapy nor a combination of surgery and chemotherapy can improve OS and CSS, suggesting that "watch and wait" may be a reasonable alternative. [ABSTRACT FROM AUTHOR]

Abstract Marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALToma) arises in extranodal sites in the head and neck. Chronic inflammatory, infectious or autoimmune conditions are implicated in its pathogenesis. Within the head and neck, MALToma is often multifocal and indolent and the imaging appearances may be mistaken for non-malignant disease in the head and neck. The aim of this article is to illustrate the varied radiological and clinical features of MALToma in the head and neck, an awareness of which is needed for timely and correct diagnosis to guide subsequent disease management.

Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) lymphoma is a type of low-grade malignant B-cell lymphoma. The aim of this study was to investigate the clinicopathological characteristics of thymic MALT lymphoma. We analyzed the clinical, morphological, immunophenotypical, cytogenetic, and molecular characteristics of 11 cases of thymic MALT lymphoma. The relevant literature was also reviewed. The median age of the 11 patients was 50 (range: 33–60). There was a female predominance with a female-to-male ratio of 10:1. Three patients presented with Sjögren syndrome, autoimmune thrombocytopenia purpura, and type B1 thymoma, respectively. Microscopically, thymic MALT lymphoma was characterized by epithelium-lined cysts that were surrounded by small lymphocytes, centrocyte-like cells, and monocytoid B-cells. Plasmacytic differentiation was observed in two cases. The tumor cells expressed CD20, CD79α, and BCL2. Clonal immunoglobulin genes were detected in all 8 examined cases. Fluorescence in situ hybridization (FISH) for 18q21 was performed in 7 cases, and no translocations involving 18q21 were found. Targeted gene sequencing was performed in five cases with available DNA samples, and TNFAIP3, CARD11, IGLL5, and CCND3 mutations were identified. Thymic MALT lymphoma is a rare type of B cell malignancy with a female predominance and excellent clinical outcomes. Molecular aberrations involving the NF-κB pathway are frequent in thymic MALT lymphoma, suggesting that dysregulation of the NF-κB pathway is an important mechanism underlying the pathogenesis of thymic MALT lymphoma. [ABSTRACT FROM AUTHOR]

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), is one of the specific type of low-grade B-cell lymphoma not infrequently found worldwide. It typically involves mucosal sites such as stomach and conjunctiva; however, primary hepatic MALT lymphoma has been extremely rarely reported. We describe a case of hepatic MALT lymphoma in a 70-year-old male patient who underwent left hepatectomy due to the incidentally detected liver masses at a medical checkup. The resected specimen revealed multinodular masses consisting of small-to-intermediate-sized lymphoid cells with serpentine pattern and focal lymphoepithelial lesions. The tumor cells were diffusely positive for CD20 and Bcl-2 but negative for CD3, CD10, CD5, CD23, CD43, and cyclinD1. The Ki-67 labeling index was 10% and immunoglobulin heavy chain gene rearrangement study confirmed monoclonal proliferation. In this paper, we discuss several unique clinicopathologic characteristics which will be helpful to the differential diagnosis of hepatic MALT lymphoma.

Mucosa-associated lymphoid tissue (MALT) is a specialized form of peripheral lymphoid tissue (LT), which is found on mucosal surfaces exposed to the environment. However, morphological data of these tissues in farm animals are scarce. This study investigated the gross anatomical and histological features of genital organ-associated lymphoid tissues (GOALTs) in the vaginal vestibule (VV) of healthy, non-pregnant, adult goats and pigs. Their VVs were composed of stratified squamous, non-keratinized epithelium, and various-sized dark-blue hematoxylin-positive spots were observed in whole-mount specimens, which were diffusely distributed throughout the mucosal surfaces. These spots were histologically identified as LTs and consisted of lymphatic nodules (LNs) or diffuse lymphoid tissue (DLTs). Both LNs and DLTs contained B cells, T cells, macrophages, dendritic cells, plasma cells, and high endothelial venules. Only the numbers of B cells were significantly higher in both the LNs and DLTs of pigs compared to goats. Furthermore, the surface of the VV epithelium covering the LTs was partially disrupted with a large intercellular space containing abundant connective tissue fibers with numerous lymphocytes. In conclusion, GOALTs in the VV appear to be common local immunological barriers in both examined animals. This knowledge is crucial for understanding the structures and disorders of female reproductive organs in farm animals.

Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis worldwide, with varied clinical and histopathological features between individuals, particularly across races. As an autoimmune disease, IgAN arises from consequences of increased circulating levels of galactose-deficient IgA1 and mesangial deposition of IgA-containing immune complexes, which are recognized as key events in the widely accepted “multi-hit” pathogenesis of IgAN. The emerging evidence further provides insights into the role of genes, environment, mucosal immunity and complement system. These developments are paralleled by the increasing availability of diagnostic tools, potential biomarkers and therapeutic agents. In this review, we summarize current evidence and outline novel findings in the prognosis, clinical trials and translational research from the updated perspectives of IgAN pathogenesis.

Mucosa-associated lymphomas of the gastrointestinal tract are a heterogeneous group differing in pathogenesis, localization and therapeutic options. For all of them, differentiated treatment requires an exact determination of lymphoma stage. For gastric MALT lymphoma, the pathogenetic role of Helicobacter pylori infection has become evident in the last 30 years. These insights were consequently implemented into clinical practice. Nowadays, Helicobacter pylori eradication is the treatment of choice for gastric MALT lymphoma, leading to complete remission of the lymphoma in the majority of cases. In the absence of success, radiotherapy is available in localized stages I/II E with excellent results. Immuno-chemotherapy is the domain for advanced stages III/IV E, and surgery plays no role any more. The rare intestinal and colorectal MALT lymphomas require an individualized therapeutic approach., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

MALT (mucosa-associated lymphoid tissue) lymphomas are low-grade extra-nodal B-cell lymphomas that may involve various sites in the head and neck including the thyroid, salivary, and lacrimal glands. Development of MALT lymphoma in the head and neck is often associated with auto-immune diseases such as Sjögren syndrome or Hashimoto thyroiditis. Here, we report a case of a MALT lymphoma of the left buucal mucosa that likely arose in the parotid gland. The patient was successfully treated with surgical excision with chemotherapy and remained disease-free at the 10-year follow-up. Since it was rare in the head and neck region, we present this case. [ABSTRACT FROM AUTHOR]

AIM: To investigate the clinical features, treatment and prognosis of primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (POAML). METHODS: A retrospective analysis was performed on 64 patients with POAML who were admitted to the First Affiliated Hospital of Zhengzhou University from January 2006 to December 2018. RESULTS: With a median follow-up of 61mo (range, 2-156mo), estimated overall survival (OS) rate and progression-free survival (PFS) rate at 10y reached 94.5% and 61.5%, respectively. Median OS time and PFS time were not reached. During this period, only 3 patients died, but none of them died directly due to disease progression. One patient (1.6%) developed transformation to diffuse large B-cell lymphoma (DLBCL). Of the 56 patients achieved complete remission after first-line treatment, 5 (8.9%) developed local and/or systemic relapse eventually. Patients ≥60y had significantly shorter PFS than younger patients (P=0.01). For patients with early stages (Ann Arbor stage I and stage II), univariate analysis confirmed that radiotherapy dose lower than 32 Gy were independently associated with shorter PFS (P=0.04). Other factors including gender, bone marrow involvement, the initial location of the disease, and the laterality were not associated with PFS. CONCLUSION: The data from our center indicate that POAML has a slow clinical progression and has an excellent clinical outcome. Patients with POAML harbor a continual risk of relaps and transformation to aggressive subtype of lymphoma.

Abstract Most patients with gastric mucosa‐associated lymphoid tissue (MALT) lymphoma are infected with Helicobacter pylori, and eradication therapy is the first‐line treatment for localized disease with H pylori infection. However, there were several reports showing effectiveness of eradication therapy in even H pylori negative cases. Gastric MALT lymphomas are endoscopically classified into three common types: superficial, ulcerative, and elevated types. For the past 20 years, we have encountered 200 cases of localized gastric MALT lymphoma. Among them, only 4 cases (2%) showed similar macroscopic findings to those of nodular gastritis (gastric MALT lymphoma with nodular gastritis‐like appearance; M‐NGA). Here, we compared clinicopathological characteristics and prevalence of non‐H pylori Helicobacter (NHPH) infection between M‐NGA and other common types of gastric MALT lymphoma. To examine the prevalence of NHPH infection, DNA was extracted from formalin‐fixed paraffin‐embedded biopsy tissues from four cases of M‐NGA, 20 cases of common endoscopic types of gastric MALT lymphoma, and 10 cases of nodular gastritis. We used a highly sensitive polymerase chain reaction assay to detect the presence of five species of NHPH (Helicobacter suis, H felis, H bizzozeronii, H salomonis, and H heilmannii). H suis infection was detected in 4, 2, and 0 of the 4, 20, and 10 cases of M‐NGA, other types of gastric MALT lymphoma, and nodular gastritis, respectively. Other NHPH species were not detected in any cases. Complete response rate by eradication therapy was 4/4 in M‐NGA cases. Therefore, nodular gastritis‐like MALT lymphoma, which shows a very rare phenotype, is closely associated with NHPH infection, and eradication therapy may be the first‐choice treatment.

The stomach is the most frequent site of extranodal lymphoma. Mucosa-associated lymphoid tissue (MALT) lymphoma is a low-grade, B-cell neoplasm strongly associated with Helicobacter pylori (HP) infection. The presenting complaints of gastric MALT lymphoma are usually nonspecific. HP eradication is regarded as the first-line therapy in early stage disease. Management of patients who failed to achieve remission following HP eradication include chemotherapy, radiotherapy and in selected cases, surgery. The aim of the present study was to examine the clinical characteristics and treatment outcome of patients with gastric MALT lymphoma.

Duodenal mucosa-associated lymphoid tissue (MALT) lymphoma is an extremely rare tumor. Herein, we report multidisciplinary treatment of a patient with synchronous development of primary MALT lymphoma of the duodenum and gastric cancer. A 70-year-old woman was referred to our hospital for examination of a gastric cancer initially diagnosed by a local medical doctor. Esophagogastroduodenoscopy showed an elevated lesion with a central ulcer in the lower body of the stomach, and a partially whitish aggregated lesion in the descending portion of the duodenum. Histopathological examination of biopsy specimens from the gastric lesion showed moderately differentiated adenocarcinoma, and duodenal specimens showed low-grade MALT lymphoma composed of atypical lymphoid cells with a lymphoepithelial lesion. The patient underwent distal gastrectomy with regional lymph node dissection for the gastric cancer. Histological examination showed muscularis propria invading adenocarcinoma with two lymph node metastases. After operation, four courses of systemic rituximab treatment were administered for the MALT lymphoma, followed by adjuvant S-1 (tegafur/gimeracil/oteracil) chemotherapy for the gastric cancer. In the 4 months after operation, the patient was well with no evidence of recurrence. To the best of our knowledge, this is the second reported case of synchronous gastric adenocarcinoma and duodenal MALT lymphoma in the English literature. [ABSTRACT FROM AUTHOR]

Abstract Pulmonary extranodal marginal zone lymphoma of the mucosa‐associated lymphoid tissue (MALT) presenting as a progressive pure ground‐glass nodule (GGN) coexisting with lung squamous cell carcinoma has not been reported. A 65‐year‐old male presented with a progressive lung GGN in the left upper lobe identified six and a half years ago but showed no symptoms. The patient had a history of tuberculosis, squamous cell carcinoma, and stomach MALT lymphoma. The patient was diagnosed with lung squamous cell carcinoma coexisting with pulmonary MALT lymphoma through computed tomography (CT)‐guided lung biopsy. A progressive lung GGN presenting in a patient with squamous cell carcinoma does not always indicate multiple primary lung adenocarcinoma, especially when given a specific medical history. The development of MALT lymphoma in the lung presenting as GGNs suggests a possible association between these two entities.

Conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma has been well-described, but rarely do these lesions demonstrate intraocular involvement. We report a case of conjunctival MALT lymphoma with intraocular involvement and novel spectral-domain ocular coherence tomography (SD-OCT) findings.A 75-year-old woman with biopsy-proven MALT lymphoma of the conjunctiva presented with ipsilateral yellowish diffuse choroidal infiltrates on fundoscopic examination. Choroidal involvement was documented clinically, on ultrasonography, on fluorescein angiography, and by SD-OCT. Treatment consisted of 3 weeks of oral doxycycline and six cycles of systemic chemotherapy with cyclophosphamide, vincristine, prednisone, and rituximab.There was no evidence of progression or recurrence of MALT lymphoma after 9 months of follow-up. Despite complete resolution of clinical findings, ultrasound, and fluorescein angiography, the choroidal lesions remained unchanged on SD-OCT.Choroidal involvement of conjunctival MALT is rare, and can be successfully treated. Persistence of irregularities on SD-OCT did not influence management in the presence of clinical improvement and resolution on ultrasonography and fluorescein angiography.

Pulmonary extranodal marginal zone lymphoma of the mucosa‐associated lymphoid tissue (MALT) presenting as a progressive pure ground‐glass nodule (GGN) coexisting with lung squamous cell carcinoma has not been reported. A 65‐year‐old male presented with a progressive lung GGN in the left upper lobe identified six and a half years ago but showed no symptoms. The patient had a history of tuberculosis, squamous cell carcinoma, and stomach MALT lymphoma. The patient was diagnosed with lung squamous cell carcinoma coexisting with pulmonary MALT lymphoma through computed tomography (CT)‐guided lung biopsy. A progressive lung GGN presenting in a patient with squamous cell carcinoma does not always indicate multiple primary lung adenocarcinoma, especially when given a specific medical history. The development of MALT lymphoma in the lung presenting as GGNs suggests a possible association between these two entities.Here, we present an extremely rare case of coexistence of lung squamous cell carcinoma with mucosa‐associated lymphoid tissue (MALT) lymphoma presenting as progressive lung ground‐glass nodules. [ABSTRACT FROM AUTHOR]

The stomach is the most frequent site of extranodal lymphoma. Mucosa-associated lymphoid tissue (MALT) lymphoma is a low-grade, B-cell neoplasm strongly associated with Helicobacter pylori (HP) infection. The presenting complaints of gastric MALT lymphoma are usually nonspecific. HP eradication is regarded as the first-line therapy in early stage disease. Management of patients who failed to achieve remission following HP eradication include chemotherapy, radiotherapy and in selected cases, surgery. The aim of the present study was to examine the clinical characteristics and treatment outcome of patients with gastric MALT lymphoma. [ABSTRACT FROM AUTHOR]

Objectives: Sjögren syndrome (SS) is considered as a major etiologic factor for primary salivary gland lymphoma (SGL). However, the percentage of SGL that is caused by SS (and thus the real impact of SS on SGL epidemiology) is unclear. We aimed to assess the prevalence of SS in patients with SGL through a systematic review and meta-analysis.Methods: Electronic databases were searched for studies assessing the presence of SS in patients with SGL. Pooled prevalence of SS in SGL was calculated, with a subgroup analysis based on histotype (mucosa-associated lymphoid tissue [MALT] vs non-MALT).Results: Sixteen studies with 665 SGLs were included. Pooled prevalence of SS in SGL was 18.2%, with high heterogeneity among studies. In MALT SGL, the prevalence of SS was 29.5%, with moderate heterogeneity. In non-MALT SGL, the prevalence of SS was 0%, with null heterogeneity.Conclusions: SS seems to be responsible for a significant but minor portion of SGLs. SS appears involved in MALT-type SGL but not in other histotypes. [ABSTRACT FROM AUTHOR]

Objectives: Lymphoid enhancer binding factor 1 (LEF1) is expressed in most cases of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and has shown utility in distinguishing CLL/SLL from other small B-cell lymphomas. LEF1 expression has not been systematically studied in CD5-positive marginal zone lymphomas (MZLs), lymphoplasmacytic lymphomas (LPLs), and follicular lymphomas (FLs). We evaluated whether these cases lacked LEF1, helping to distinguish them from CLL/SLL.Methods: MZLs, LPLs, and FLs expressing CD5 were retrospectively studied for expression of LEF1 by immunohistochemistry.Results: LEF1 was absent in 17 of 18 CD5-positive lymphomas including 13 MZLs (2 nodal, 3 splenic, and 8 mucosa-associated lymphoid tissue lymphomas), 3 LPLs, and 1 of 2 FLs. One grade 3A CD5-positive FL expressed LEF1 in a majority of tumor cells.Conclusions: LEF1 is not expressed in most CD5-positive MZLs and LPLs; therefore, it is a reliable marker for distinguishing them from CLL/SLL. LEF1 may be expressed in CD5-positive FLs. [ABSTRACT FROM AUTHOR]

Objectives: To assess the prevalence of Chlamydia psittaci, Chlamydia pneumoniae, and Chlamydia trachomatis in ocular adnexa lymphoma (OAL) determined by molecular testing in different countries and the potential association of Chlamydia infection with mucosa-associated lymphoid tissue (MALT) histotype by performing a systematic review and meta-analysis.Methods: Electronic databases were searched for studies assessing the presence of Chlamydia in OAL. Pooled prevalence of the three Chlamydia species was calculated in each country. An odds ratio was calculated for the association between Chlamydia and MALT histotype, with a significant P < .05.Results: Thirty-seven studies with 1,188 OALs were included. Pooled prevalence of C psittaci, C pneumoniae, and C trachomatis by country was done. Chlamydia infection was significantly associated with MALT histotype (odds ratio, 2.183; P = .027).Conclusions: The involvement of C psittaci in OAL is highly variable, with the highest prevalence in Italy and Korea. Chlamydia is associated with MALT histotype. [ABSTRACT FROM AUTHOR]